Tumoral, quasitumoral and pseudotumoral lesions of the ... - Siapec

PATHOLOGICA 2006;98:239-298 

CONOSCERE PER RICONOSCERE 

THE EYE WILL RECOGNIZE WHAT THE MIND ALREADY KNOWS 

UPDATING ARTICLE IN SOFT TISSUE PATHOLOGY 

ARTICOLO DI AGGIORNAMENTO IN PATOLOGIA DELLE PARTI MOLLI 

Tumoral, quasitumoral and pseudotumoral lesions of 

the superficial and somatic soft tissue: new entities 

and new variants of old entities recorded during the 

last 25 years. Part XII: Appendix 

Lesioni tumorali, quasitumorali e pseudotumorali delle parti molli somatiche e 

superficiali: entità nuove e varianti di entità già note, descritte negli ultimi 25 

anni. Parte XII: Appendix 

M. BISCEGLIA, D. SPAGNOLO 1 , C. GALLIANI 2 , C. FISHER 3 , S. SUSTER 4 , D.V. KAZAKOV 5 , K. COOPER 6 , M. MICHAL 5 

Department of Pathology, IRCCS “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, Italy; 1 PathWest Laboratory Medicine 

WA, Nedlands, Western Australia, Australia; 2 Department of Pathology, Cook Children’s Medical Center, Fort Worth, Texas, USA; 

3 

Department of Histopathology, Royal Marsden Hospital, London, United Kingdom; 4 Department of Pathology, Ohio State University 

Hospitals, Columbus, OH, USA; 5 Department of Pathology, Medical Faculty Hospitals, Charles University, Pilsen, Czech Republic; 

6 Department of Pathology, University of Vermont/Fletcher Allen Health Care, Burlington, VT, USA 

Summary 

In an eleven part series published in Pathologica, we have presented 

various tumoral, quasitumoral and pseudotumoral lesions 

of the superficial and somatic soft tissue (ST), which emerged as 

new entities or as variants of established entities during the last 

quarter of a century. Detailed clinicomorphological and differential 

diagnostic features of approximately sixty entities were 

chosen on the basis of their clinical significance and morphologic 

distinctiveness. The series included fibrous and myofibroblastic 

tumors (e.g. solitary fibrous tumor, high grade classic and pigmented 

dermatofibrosarcoma protuberans, inflammatory myofibroblastic 

tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic 

neoplasms (e.g. Evans’ tumor, phosphaturic mesenchymal 

tumor, inflammatory myxohyaline tumor), special 

adipocytic / vascular / and smooth muscle lesions (e.g. chondroid 

lipoma, Dabska’s tumor, ST hemangioblastoma, lipoleiomyosarcoma), 

epithelioid mesenchymal malignancies of diverse lineages 

(e.g. epithelioid liposarcoma, proximal-type epithelioid sarcoma, 

neuroendocrine extraskeletal chondromyxoid sarcoma), ST 

Ewing’s tumor and peripheral nerve sheath tumors (perineuriomas 

and pigmented and rosetting tumors of the schwannoma/neurofibroma 

group), extranodal dendritic or histiocytic proliferative 

processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, 

Castleman’s disease, and plexiform xanthomatous tumor), 

and tumors with myoepithelial differentiation. The section 

devoted to selected pseudotumoral entities considered representatives 

of the hamartoma group (neural fibrolipomatous hamartoma, 

ectopic hamartomatous thymoma, rudimentary meningocele), 

metabolic diseases (amyloid tumor, nephrogenic fibrosing 

dermopathy, tophaceous pseudogout, pseudoinfiltrative parathyromatosis), 

stromal tissue reactions to trauma (fibroosseous 

pseudotumors of digits) and infections (bacillary angiomatosis), 

and normal organs (glomus coccygeum). 

To conclude the descriptive phase, supplementary material has 

now been collected and appended in an attempt to provide a 

quick digest of essential knowledge both for comparison and 

differential diagnosis. The data have been tailored to synthesize 

diverse sources, integrating clinical elements and references to 

articles that previously appeared in Part I (“Introduction”), Part 

II (“The List and Review of New Entities”) and Parts III to XI 

(“Excerpta”). At the very least we hope this final part (“Appendix”) 

will provide the reader with a useful tabular organization 

of ST lesions and a reference resource. 

Introduction 

In 2003 Dr Bisceglia was commissioned by the Editors 

of Pathologica to produce a series of articles concerning 

tumoral, quasitumoral or pseudotumoral lesions of superficial 

and somatic soft tissue recognised in the last 

quarter century, including newly described entities and 

new variants of established entities. Through a series of 

11 articles published in the journal 1-11 , Dr Bisceglia and 

colleagues have presented both a global overview of the 

numerous entities which have been described in this period, 

with few exceptions dating from 1979, and also 

Corrispondenza 

Michele Bisceglia, M.D., Unità Operativa Anatomia Patologica, 

IRCCS- Ospedale “Casa Sollievo della Sofferenza”, 71013 San 

Giovanni Rotondo (FG), Italy - Tel. +39 0882 410366 - Fax +39 

0882 410411 - E-mail: bismi@libero.it

240 

M. BISCEGLIA ET AL. 

provided detailed discussion, including any novel data, 

of selected entities. This information was presented in a 

logical and algorithmic manner, both in extensive tabular 

and discursive formats, supported by a comprehensive 

but selected, and up-to-date bibliography. This allows 

the reader to navigate through the vast amount of information 

which has accrued in the area of soft tissue 

pathology in the last 25 years or so. The intent, and its 

desired result, is that this treatise should be a valuable resource 

not only for pathologists who regularly encounter 

soft tissue lesions in their practice, but more importantly 

for those who navigate the diagnostic soft tissue traps on 

a more sporadic basis, for residents and fellows in histopathology, 

and clinicians dealing with soft tissue lesions. 

A brief summary of the approach used in this series of 

articles is presented here to facilitate retrospective navigation 

through the series. 

The first article (in Italian) 1 provides an introduction 

and raison d’être for the project, criteria for categorization 

and a detailed reasoning for inclusion or exclusion 

of various conditions. It ends with a “master table” 

of diagnostic categories, presented in 2 broad groupings 

of “Tumoral and Quasitumoral Lesions” (18 diagnostic 

categories) and “Pseudotumoral Lesions” (10 

diagnostic categories). This Table forms the backbone 

for the subsequent articles and excerpta which expand 

in detail on many, but not all lesions which are included 

in each diagnostic category. To compile the comprehensive 

catalogue of lesions considered in the subsequent 

articles in this series, the authors have drawn 

not only on an exhaustive literature review (which includes 

peer-reviewed articles and important discussions 

appearing as letters to the Editor), but also the 

considerable personal experience of many of them in 

the field of soft tissue pathology. The authors have 

wherever possible listed entities according to their presumed 

or accepted histogenesis/differentiation, while a 

category is retained for those of uncertain histogenesis/differentiation. 

The rationale for certain morphological 

groupings is provided, for example, those defined 

on the basis of a distinctive extracellular matrix (e.g. fibromyxoid 

tumors), or cellular composition (e.g. fibrohistiocytic 

or fibromyofibroblastic tumors). 

The second article 2 , prefaced with a preamble in Italian, 

presents the comprehensive list of entities (in English) 

which fall within each diagnostic category, accompanied 

by key references. The latter include important 

references of a general or review nature in respect 

of particular diagnostic categories/entities, in addition 

to references specific to particular entities and their variants. 

While all of the diagnostic categories of the 

2002 WHO classification of soft tissue tumors 12 13 are 

included in the master table, by necessity and design, 

even within the categories of tumorous lesions, there 

has been a deliberate “splitting” so that in contrast to 

the WHO scheme, some categories appear in their own 

right as major diagnostic categories, e.g. the myoepithelial 

tumors, malignant mixed mesenchymal tumors 

and neoplasms of perivascular epithelioid cell 

origin. It is the authors’ intention that the “exploded” list 

of entities with their pertinent bibliography can provide 

the casual reader, as well as a pathologist dealing 

with a specific soft tissue problem, an intuitive and rapid 

means of assessing the breadth of differential diagnostic 

possibilities and the state of knowledge that 

exists at the time of publication. Selected entities to be 

discussed in clinicopathologic detail and illustrated in 

the subsequent articles (“Excerpta”) are highlighted in 

the list. They were selected for detailed discussion on 

the basis of their clinical importance and/or their distinct 

morphological features. 

The third to eleventh articles in the series (Excerpta 1 

to 9) 3-11 are devoted to presenting the clinicopathologic 

and biologic features of specific entities (58 in all) in 

detail. The following Table summarizes the entities 

which are discussed in depth in each Excerpta.

TUMORAL, QUASITUMORAL AND PSEUDOTUMORAL LESIONS OF SOFT TISSUE - APPENDIX 241 

Series article #/ Ref. # Entities presented in detail Author Pathologica 

Excerpta # (initials) fascicle 

Part 3 / Excerpta 1 3 1. Dermatofibrosacoma protuberans, classic, and pigmented sarcomatous MB GIUGNO 

2. Atypical fibroxanthoma, non-pleomorphic spindle-cell, and aneurysmal (pigmented) MB (June) 

3. Plexiform fibrohistiocytic tumor KC 2004 

4. Hemosiderotic fibrohistiocytic lipomatous lesion DVK 

5. Nuchal-type fibroma MM 

6. Desmoplastic fibroblastoma CF 

7. Solitary fibrous tumor SS 

8. Pleomorphic hyalinizing angiectatic tumor SS 

9. Sclerosing epithelioid fibrosarcoma KC 

Part 4 / Excerpta 2 4 1. Dermatomyofibroma MB OTTOBRE 

2. Palisaded myofibroblastoma / intranodal hemorrhagic spindle cell tumor with SS (October) 

“amianthoid” fibres 2004 

3. Myofibrosarcoma, low grade and high grade CF 

4. Angiomyofibroblastoma of vulva DVK 

5. Lipomatous hemangiopericytoma CF 

6. Inflammatory pseudotumor / inflammatory myofibroblastic tumor MB 

7. Calcifying fibrous (pseudo)tumor MB 

8. Inflammatory myxohyaline tumor / myxoinflammatory fibroblastic sarcoma MM 

9. Superficial angiomyxoma and juxtarticular myxoma KC 

10. Ossifying fibromyxoid tumor CF 

11. Low grade fibromyxoid sarcoma & hyalinising spindle cell tumor with giant rosettes KC 

Part 5 / Excerpta 3 5 1. Phosphaturic mesenchymal tumor MB DICEMBRE 

2. Chondroid lipoma DVK (December) 

3. Dendritic fibromyxolipoma SS 2004 

4. Epithelioid liposarcoma KC 

5. Lipoleiomyosarcoma SS 

6. Epithelioid leiomyosarcoma SS 

7. Granular cell leiomyosarcoma SS 

Part 6 / Excerpta 4 6 1. Acquired elastotic hemangioma DVK FEBBRAIO 

2. Epithelioid hemangioendothelioma of soft tissue and skin MB (February) 

3. Extraneuraxial hemangioblastoma of soft tissue and skin MM 2005 

4. Dabska’s tumor arising in lymphangioma MB 

Part 7 / Excerpta 5 7 1. Psammomatous melanotic Schwannoma KC APRILE 

2. Pigmented neurofibroma MM (April) 

3. Dendritic cell neurofibroma with pseudorosettes DVK 2005 

4. Epithelial sheath neuroma DVK 

5. Perineuriomas - intraneural and extraneurial forms MB 

6. Ewing’s sarcoma of the skin MB 

Part 8 / Excerpta 6 8 1. Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation CF GIUGNO 

2. Proximal epithelioid sarcoma CF (June) 

3. Extra-renal malignant rhabdoid tumor CF 2005 

4. Follicular dendritic cell sarcoma CF 

5. Castleman’s disease of the subcutis and skeletal muscle DVK 

6. Rosai-Dorfman disease of soft tissue KC 

7. Plexiform xanthomatous tumor MM 

Part 9 / Excerpta 7 9 1. Hyaline cell-rich chondroid syringoma and myoepithelial tumors - mixed tumor and MB OTTOBRE 

myoepithelioma 

(October) 

2. Dabska’s parachordoma MM 2005 

3. Oncocytoma MB 

Part 10 / Excerpta 8 10 1. Ectopic hamartomatous thymoma DVK DICEMBRE 

2. Hamartoma of the scalp with ectopic meningotheliomatous elements / rudimentary DVK (December) 

meningocele 2005 

3. Neural fibrolipomatous hamartoma MB 

4. Ischemic fasciitis (atypical decubital fibroplasia) MM 

5. Nephrogenic fibrosing dermopathy DVK 

6. Bacillary epithelioid angiomatosis KC 

Part 11 / Excerpta 9 11 1. Fibro-osseous pseudotumor of the digits and bizarre parosteal osteochondromatous MB GIUGNO 

proliferation (Nora’s lesion) 

(June) 

2. Tophaceous pseudogout (calcium pyrophosphate dihydrate crystals deposition MB 2006 

disease – tumoral form) 

3. Amyloid tumor of soft tissue and breast MB 

4. Glomus coccygeum MB 

5. Post-implant parathyromatosis MB

242 


In this final APPENDIX (Part XII) to the series, 30 

“problem-oriented” Tables (with bibliography) are 

provided to aid the decision making process in everyday 

diagnostic practice in soft tissue pathology. These 

Tables provide a comprehensive, though admittedly 

not exhaustive grouping of lesions according to 

important clues based on cytoarchitectural pattern recognition, 

and also in consideration of particular clinical 

circumstances. The Tables should serve not 

only as an aide-de-memoir but also as a diagnostic 

“check-list” when confronted with a particular diagnostic 

problem. To provide maximum benefit from 

this problem-oriented tabular approach, we thought 

that it would be useful to broaden the scope of the tables 

beyond those lesions affecting superficial and 

somatic soft tissues, as was our original intent in the 

series, to also include lesions affecting other anatomic 

parts/organs, e.g. the body cavities. However, genuine 

soft tissue visceral organ-based lesions have 

been excluded from the analysis. Updated references 

for various entities considered in the preceding Excerpta 

are provided, in addition to important reviews 

and references of a more generic nature. References 

to the new entities which had been already quoted in 

the General List of entities (Part II) or were detailed 

in the Excerpta – with very few exceptions limited to 

Table 30 – do not appear in this Appendix, to avoid 

repetition. Entities which have been previously described 

or referenced are indicated by means of various 

typographical options. 

The Key to navigating the Tables is as follows. 

• Bolded entities appeared in Excerpta and are listed 

alphabetically in the Index at the end of this Appendix, 

accompanied by key references that have appeared 

since the publication of the excerpta. 

• Italicized entities were listed in Part II of the Series 

2 

with their annotated bibliography. 

• Referenced entities in the Tables correspond to recent 

articles that appeared after Part II of our series 

was published 2 , or were not included in the previous 

List, or are considered to be key and/or review 

articles. 

To keep abreast of the burgeoning growth in knowledge 

of soft tissue tumefactive conditions can be 

overwhelming, and to mine the breadth and depth of 

the data base excerpted from the eleven preceding publications 

requires an agility that can only be met by 

the power of online search engines. Since the publication 

of the series in Pathologica, outstanding contributions 

have appeared in the world literature 14 15 . In this 

Appendix we broaden our previous list of entities and 

update the bibliography. To complement the traditional 

morphologic approach used throughout the series, the 

reader is referred to the January 2006 issue of Histopathology, 

which is devoted to soft tissue tumors and 

which highlights the increasing role of new diagnostic 

modalities, such as genetic analysis and microarray technology 

16-18 , in addition to the traditional contributions 

of immunohistochemical and ultrastructural examination 

19 . Continued refinement in nosology is possible 

through advances in the expertise of the investigator 

and the diagnostic tools being applied. Histological 

classifications and subclassifications that are practically 

achieved, reproducible and uniquely predictive, 

either alone or in conjunction with other patient’s characteristics, 

are valuable in the design and evaluation of 

therapeutic regimens. It is our hope that the series with 

its comprehensive Appendix will provide the reader 

with a rapid reference base, maximum availability, and 

practical usefulness.


“Problem-oriented” tables 

With the intent to allow one, at a glance, to be pointed in the right direction, and hopefully provoke a highly satisfying 

“Eureka” moment!! 

1. Richly collagenized lesions 

A. Tumor-like lesions 

B. Tumoral lesions 

2. Myxoid lesions 

A. Myxomatous lesions 

B. Occasionally myxomatous lesions, or with myxomatous change 

3. Tumors and pseudotumors with calcifications, or with chondro-osseous metaplasia 

A. Dystrophic and psammomatous calcifications 

B. Chondroid and osseous metaplasia 

4. Tumors and pseudotumors with inflammatory cell component 

A. Lymphoid infiltrate 

B. Xanthomatous infiltrate 

5. Atypical / bizarre / multinucleated / pleomorphic giant cell-containing tumors and pseudotumors 

A. Atypical / bizarre / monster cells in benign lesions 

B. Reactive multinucleated, osteoclast-like giant cells in benign tumors and pseudotumors 

C. Osteoclast-like giant cells in borderline and malignant tumors 

D. Multinucleated giant cells (intrinsic) in benign tumors 

E. Neoplastic giant cells (intrinsic or accompanying) in borderline and malignant tumors 

F. “Malignant fibrous histiocytoma”-like pattern 

6. Tumors and pseudotumors with a distinctive architecture / growth pattern 

A. Fascicular pattern 

B. Storiform pattern 

C. Whorled pattern 

D. Palisading pattern 

E. Amianthoid structures and (pseudo-)rosettes 

F. Cording / chordoid pattern 

G. Endocrinoid pattern 

H. Alveolar pattern 

I. Pseudoglandular pattern 

J. Pseudovascular pattern 

K. Retiform pattern 

L. Papillary pattern 

7. Distinctive vascular patterned lesions (non-endothelial) 

A. Hemangiopericytoma-like pattern as a constant feature 

B. Hemangiopericytoma-like pattern as an occasional feature 

C. Perivascular hyalinization prominent 

D. Plexiform capillary pattern 

E. Glomeruloid capillary pattern 

F. Hemorrhagic changes / pattern 

G. Aneurysmal changes / pattern 

8. Combined, polyphasic, and heterologous tumors (other than collision tumors) 

A. Combined (homologous and heterologous) tumors 

B. Biphasic homologous (bimorphic) tumors 

C. Biphasic heterologous tumors – with epithelial elements 

D. Pseudo-biphasic tumors 

E. Biphasic tumors with skeletal muscle differentiation 

9. Histogenetically diverse but morphologically similar tumors and pseudotumors with …. 

A. Round-cell / epithelioid / plasmacytoid features 

B. Clear cell changes 

C. (Uni-/multi-)vacuolated cells, (pseudo-)lipoblasts, and (pseudo-)physalipherous cells 

D. Eosinophilic / oncocytic features 

E. Granular cells 

F. Rhabdoid / pseudo-rhabdoid features (extra-renal) 

G. Ganglion / ganglion-like cells 

H. Small round cells 

I. Pleomorphic cells

244 


10. Tumors and pseudotumors with diagnostic clues 

A. Architecturally distinct features 

B. Stromal peculiarities 

C. Cyto-histologically distinct features 

11. Histologically pigmented tumors and pseudotumors with …. 

A. Melanin 

B. Hemosiderin 

C. Carbon 

12. Clinically alarming benign tumors and pseudotumors 

A. Multiple, multicentric, recurring benign lesions 

B. Painful soft tissue tumors and pseudotumors 

13. Pathologically alarming benign tumors and pseudotumors 

A. Multilobular / mosaic growth pattern 

B. Plexiform, infiltrating margins 

C. Intravascular growth 

D. Hypercellular benign lesions 

14. Histologically benign lesions mistaken for malignant 



15. Reactive endothelial lesions that may histologically mimic malignancy 

16. Histologically malignant tumors mistaken for benign lesions 

17. Atypical variants of typical tumors 

A. Clinically atypical 

B. Behaviourally atypical 

C. Macroscopically atypical 

D. Morphologically atypical (histologic/cytologic) 

18. Cystic tumors and pseudotumors 

A. Primarily cystic 

B. Secondarily cystic or with microcystic change 

C. With pseudocystic pattern 

19. Acral tumors and pseudotumors 

20. Orbital tumors and pseudotumors 

21. Paratesticular tumors and pseudotumors 

A. Benign tumors and pseudotumors 

B. Malignant tumors 

22. Vulvo-vaginal tumors and pseudotumors 

23. Aerodigestive tract lesions (involving the orifices) 

A. Nasal cavity 

B. Oral cavity 

C. Anal canal 

24. Intranodal primary tumors and pseudotumors (other than hematolymphoid) 

25. Mammary stromal spindle cell tumors and tumor-like lesions and mimics 

A. Monomorphic spindle cell tumors of specialized mammary stroma 

B. Monomorphic spindle cell tumors of non-specialized mammary stroma 

C. Spindle cell myoepithelioma 

D. Spindle cell epithelial tumors 

26. Infantile-juvenile tumoral and pseudotumoral lesions 

A. Hamartomas – Developmental lesions 

B. Benign tumors and pseudotumors 

C. Intermediate and malignant tumors 

27. Lesions as sentinel of clinical entities 

28. Lesions as sentinel of clinical syndromes 

29. Embryological rests or normal anatomic structures potentially mistaken for tumors 

30. Grade-based categorization of soft tissue tumors (of relevance to management)


Table I. Richly collagenized lesions 20 . 


• Fibrous papule of the face (nose) (classic type and variants) 21 

• Multiple perifollicular fibroma of the face and neck 

• Angiofibromas (associated and unassociated with tuberous sclerosis complex) 

• Collagenoma (connective tissue nevus) 

• Rudimentary supernumerary digit 

• Acquired digital fibrokeratoma 

• Sclerotic fibroma (circumscribed storiform collagenoma) 

• Reactive fibrosis 

• Hypertrophic scar & keloid 

• Knuckle pads 

• Diabetic scleredema (upper back) 

• Elastofibroma 

• Infantile digital fibromatosis 

• Gingival fibromatosis 

• Juvenile hyaline fibromatosis 22 

• Fibrous proliferations in Proteus syndrome 

• Intra-articular nodular fasciitis 23 

• Calcifying fibrous pseudotumor 

• Idiopathic retroperitoneal fibrosis (Ormond’s disease) 

24, 25 

• Reactive nodular fibrous pseudotumors (of the gastrointestinal tract and mesentery) 

• Sclerosing mediastinitis 25a 

• Sclerosing extramedullary hematopoietic tumor 


• Nasopharyngeal angiofibroma 

• Sclerosing/desmoplastic melanocytic nevi (ordinary acquired melanocytic nevus, Spitz nevus, blue nevus) 

• Dermatofibroma variant (keloidal, sclerotic) 

• Pleomorphic fibroma 

• Nuchal-type fibroma (of nuchal and extra-nuchal sites) 

• Gardner-associated fibroma 

• Fibroma of tendon sheath 

• Giant cell fibroblastoma 

• Sclerotic lipoma 

• Fibrous spindle cell lipoma 

• Sclerosing neurofibroma 

• Desmoplastic cutaneous ganglioneuroma 

• Atypical fibroxanthoma with prominent sclerosis 

• Tenosynovial giant cell tumor – sclerotic variant 

• Desmoplastic fibroblastoma (collagenous fibroma) 

• Sclerosing perineurioma 

• Superficial (fascial) fibromatosis (palmar / plantar / penile types) 

• Deep (musculoaponeurotic) fibromatosis (desmoid tumors: extraabdominal / abdominal / intraabdominal types) 

• Paratesticular fibroma 

• Solitary fibrous tumor 26 

• Epithelioid hemangioendothelioma 

• Sclerosing epithelioid fibrosarcoma 

• Sclerosing primary cutaneous leiomyosarcoma 

• “Fibroma-like” epithelioid sarcoma (- desmoplastic variant) 26a 

• Sclerosing rhabdomyosarcoma 

• Sclerosing paraganglioma 27 

• Monophasic synovial sarcoma 

• Leiomyomatosis peritonealis disseminata (end-stage) 

• Desmoplastic diffuse mesothelioma 

• Desmoplastic small round cell tumor 

• Desmoplastic melanoma 27a 

• Lymphoma (occasional)

246 


Table II. Myxoid lesions 28 28a . 

A. Myxomatous lesions 

• Focal mucinosis (cutaneous and subcutaneous) 29 

• Polyvynilpyrrolidone granuloma (rare: may occur in the skin/subcutis/soft tissue/bone) 

• Superficial angiomyxoma (cutaneous and subcutaneous myxoma) & juxta-articular myxoma 

• Neurothekeoma, classic or myxoid type 

• Nerve sheath myxoma (“perineurial myxoma” of Harkin and Reed – “plexiform myxoma”) 

• Myxolipoma (myxoid lipoma) 

• Angiomyxolipoma 

• Dendritic fibromyxolipoma / Myxoid spindle cell lipoma 

• Intramuscular myxoma 

• Myxopapillary ependymoma 

• Superficial acral fibromyxoma 

• Aggressive angiomyxoma 

• Ossifying fibromyxoid tumor 

• Low-grade fibromyxoid sarcoma (Evans’ tumor) 

• Superficial low-grade fibromyxoid sarcoma (Evans’ tumor) 30 

• Primitive myxoid mesenchymal tumor of infancy 31 

• Myxofibrosarcoma (low grade myxoid malignant fibrous histiocytoma) 32 

• Extraskeletal myxoid chondrosarcoma (chordoid sarcoma) 

• Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation 

• Myxoid liposarcoma 

• Primary heterotopic chordoma 28 28b , chordoma periphericum 32a 

32b 32c 

• Parachordoma 

• Mixed tumors of salivary glands (recurrence in soft tissue) 

• Metastatic myxoid sarcomas to soft tissue and skin 33 

• Metastatic chordoma 

• Metastatic colloid carcinoma 

• Pseudomyxoma peritonei 

B. Lesions occasionally myxomatous or with myxomatous change 

• Trichodiscoma (fibrofolliculoma) 

• Keloid 

• Nodular fasciitis 

• Infantile fibromatosis 

• Subungual pleomorphic fibroma – myxoid variant 

• Proliferative funiculitis 

• Fibrous umbilical polyp 

• Cutaneous myxoid fibroblastoma 


• Dermatofibrosarcoma protuberans – myxoid variant 

• Myoepithelioma 

• Lipoblastoma 

• Leiomyoma 

• Schwannoma – myxoid variant 

• Neurofibroma – myxoid variant 

• Dendrocytoma –myxoid variant 

• Chondroma – myxoid variant 

• Fetal-type rhabdomyoma 

• Spindle cell lipoma (occasional) 

• Perineurioma – myxoid variant 

• Dermatofibrosarcoma protuberans – myxoid variant 

• Solitary fibrous tumor – myxoid variant 

• Leiomyosarcoma – myxoid variant 

• Malignant peripheral nerve sheath tumor 

• Rhabdomyosarcoma (mainly botryoid embryonal type) 

• Inflammatory acral myxo-hyaline fibroblastic sarcoma 

• (Extra-)gastrointestinal stromal tumor 33a 

• Fibrous synovial sarcoma 

• Clear cell sarcoma – myxoid variant 34 

• Lymphoma (occasional) 

• Melanoma (primary, and metastatic) – myxomatous 35 36 [and pseudomyxomatous 36a ] variants


Table III. Tumors and pseudo-tumors with calcifications, or with chondro-osseous 

metaplasia. 

A. Tumors and pseudo-tumors with dystrophic and psammomatous calcifications 

• Hemangiomas (phleboliths mainly in cavernous and spindle cell hemangioma) 

• Cutaneous meningioma 

• Amyloid tumor (even bone) 

• Tophaceous gout (even bone) 

• Tophaceous pseudogout (tumoral crystal calcium pyrophosphate dihydrate deposition disease) 

• Malakoplakia 

• Fat necrosis 

• Calcinosis – several types 

• Calcifying aponeurotic fibroma 

• Myofibroma/myofibromatosis 

• Calcifying fibrous pseudotumors 

• Leiomyoma 

• Neurothekeoma 

• Ancient schwannoma 

• Phosphaturic mesenchymal tumor (mixed connective tissue variant) 

• Hyalinizing spindle cell tumor with giant rosettes 

• Ganglioneuroma 

• Psammomatous melanotic schwannoma 

• Psammomatous malignant melanoma 37 

• Epithelioid sarcoma 

• Mesothelioma 

• Synovial sarcoma 

• Differentiating neuroblastoma 

• Alveolar soft part sarcoma with psammomatous bodies, and calcifications 37a (occasional) 

• Any sarcoma with preceding necrosis (may undergo dystrophic calcification) 

• Extragonadal (retroperitoneal) burnt-out germ cell tumors 

B. Soft tissue tumors and pseudotumors with chondroid & osseous metaplasia 38 

• (Pilomatrixoma) 

• Ectopic ossification of the auricle secondary to frostbite 

• Nuchal fibrocartilaginous pseudotumor 

• Heterotopic chondro-ossification 

• (Chondro-)mesenchymal hamartoma of the nose 39 

• Mesenchymal hamartoma of the chest wall 40 

• Fibrochondroid hamartoma 

• Rhabdomyomatous mesenchymal hamartoma of the skin (occasional) 

• Hyperplastic callus in pathologic conditions (e.g. osteogenesis imperfecta, …) 

• Tumefactive soft tissue extension of Paget’s pseudosarcoma 

• Cavernous hemangioma with ossification (occasional) 

• Dermatofibroma (ossifying variant) 

• Benign fibrous histiocytoma of subcutaneous and deep soft tissue (occasional) 

• Fibromatosis (mainly palmar type) 

• Juvenile xanthogranuloma (1 case with ossification) 

• Myofibromatosis 

• Epithelioid hemangioendothelioma with ossification 

• Cutaneous chondroma (solitary form, multiple / familial form) 

• Cutaneous osteoma 

• Chondro-osteo-lipoma / benign mesenchymoma 

• Hyaline cell-rich chondroid syringoma 

• Chondroid lipoma 

• Soft tissue chondroma & periosteal chondroma 

• Extra-articular synovial chondromatosis 40a 

• Nodular fasciitis, mainly parosteal variant (fasciitis ossificans) 

• Myositis & panniculitis ossificans 

• Heterotopic mesenteric ossification 41 

• Subungual (Dupuytren's) exostosis / turret exostosis (acquired osteochondroma) of the hands and feet 

• Fibroosseous pseudotumor of the digits (florid reactive periostitis of the tubular bones of the hands and 

feet)

248 


• Florid reactive periostitis of the long bones (e.g. tibia 41a ) 

• Bizarre parosteal osteochondromatous proliferation of the hands and feet (Nora's lesion) 

• Bizarre parosteal osteochondromatous proliferation of the long bones (Nora's lesion) 

• Fibrodysplasia ossificans progressiva 

• Ossified meningioma 


• Atypical fibroxanthoma with osteoid production 

• Atypical fibroxanthoma with chondroid differentiation 

• Mixed tumor of the skin and soft tissue 

• Giant cell tumor of tendon sheath & giant cell tumor of soft tissue 

• Phosphaturic mesenchymal tumor (mixed connective tissue variant) 

• Hemangiopericytoma 

• Schwannoma with bone or cartilage 


• Malignant fibrous histiocytoma 

• Epithelioid sarcoma (with chondroid and osseous metaplasia) 


• Embryonal rhabdomyosarcoma 

• Extraskeletal myxoid chondrosarcoma (chordoid sarcoma) 


• Extraskeletal soft tissue & synovial chondrosarcoma 

• Soft tissue mesenchymal chondrosarcoma 

• Periosteal chondrosarcoma 

• Parosteal, periosteal, high-grade surface osteosarcoma 

• Soft tissue extraskeletal osteosarcoma 

• Primary cutaneous osteosarcoma 

• Primary osteogenic sarcoma of the penis 

• Primary osteogenic sarcoma of the breast 

• Malignant mesenchymoma 

• Mesothelioma (occasional) 

• Extraskeletal PNET (with massive osteocartilaginous differentiation) 

• Melanoma (osteogenic melanoma) 42 

• Metastatic metaplastic breast carcinoma, malignant mixed mullerian tumor, skeletal and extraskeletal osteogenic 

sarcoma


Table IV. Tumors and pseudotumors with inflammatory cell component. 

A. Tumors and pseudotumors with lymphoid infiltrate 

• Inflammatory fibrous papule of the face 21 

• Lymphangioma 

• Amyloid tumor 

• Atypical mycobacterial spindle cell pseudotumor 

• Pseudoneoplastic (histoid) leprosy 


• Bacillary angiomatosis 

• Angiolymphoid hyperplasia with eosinophilia 

• Kimura’s disease 

• Lymphangioma-like Kaposi’s sarcoma 

• “Lymphocyte-rich hobnail hemangioendothelioma” (including retiform hemangioendothelioma 

and Dabska’s tumor) 

• Rosai-Dorfman disease, extranodal 

24 25 


• Sclerosing mesenteritis, mesenteric panniculitis, mesenteric lipodystrophy, and other inflammatory 

43 44 

pseudotumors 

• Sclerosing retroperitonitis (Ormond’s disease) 

• Sclerosing mediastinitis (idiopathic fibroinflammatory lesions of the mediastinum) 25a 

• Inflammatory pseudotumor (inflammatory myofibroblastic tumor) 

• “Inflammatory fibrosarcoma” 

• Inflammatory liposarcoma (lymphocyte-rich well differentiated liposarcoma) 

• Inflammatory leiomyosarcoma 

• Inflammatory malignant fibrous histiocytoma / undifferentiated pleomorphic sarcoma with prominent 

inflammation 

• Malignant fibrous histiocytoma – pseudolymphomatous variant 

• Angiomatoid fibrous histiocytoma 

• Acral inflammatory myxohyaline tumor (myxoinflammatory fibroblastic sarcoma) 

• Dedifferentiated liposarcoma – pseudolymphomatous variant 45 

• Diffuse-type giant cell tumor 

• Mesothelioma (lymphohistiocytoid variant) 

• Follicular dendritic cell sarcoma 

B. Tumors and tumor-like lesions with xanthomatous infiltrate 

• Xanthomatous leprosy 

• Atypical mycobacteriosis 

• Nodular panniculitis 

• Nodular-cystic encapsulated fat necrosis (“encapsulated mobile lipoma”) 

• Paraffinoma / oleogranuloma (other than lung paraffinoma and coeliac lipogranulomatous lymphadenitis) 

• Lipoma with fat necrosis 

• Juvenile xanthogranuloma 

• Necrobiotic xanthogranuloma 

• Xanthomatous / cholesterolotic benign fibrous histiocytoma 

• Xanthoma (tuberous type) 

• Plexiform xanthomatous tumor 

• Xanthogranulomatous inflammation NOS (of any site, including abdomen and pelvis) 

• Pelvic xanthogranulomatous inflammation of infective etiology – overt (e.g. actinomyces) or occult 46 

47 48 

• Tamm-Horsfall protein deposition (early phases) 

• Erdheim-Chester disease with xanthogranulomatous inflammation 

• Erdheim-Chester of the breast 49 

• Tenosynovial giant cell tumor both localized and diffuse-type 

• Schwannoma 

• Malignant fibrous histiocytoma – inflammatory type

250 


Table V. Atypical / bizarre / multinucleated / pleomorphic giant cell-containing 

tumors and pseudotumors. 

A. Benign tumors with atypical / bizarre / monster cells 

• Pleomorphic fibroma of the skin 

• Subungual pleomorphic fibroma 

• Pleomorphic fibroma of tendon sheath 

• Pleomorphic lipoma 

• Pleomorphic leiomyoma 

• Atypical decubital fibroplasia of debilitated people (ischemic fasciitis) 

• Orbital & extraorbital giant cell angiofibro(blast)oma 

• Pseudosarcomatous benign fibrous histiocytoma 

• Atypical lipoma 


• Paraganglioma 

• Neurofibroma with nuclear atypia 

• Symplastic glomus tumor 

• Symplastic leiomyoma 

• Bizarre angioleiomyoma 

• Atrophic striated muscle cells in several conditions 

• Regenerating skeletal muscle 

• Pseudosarcomatous polyp 

• Cellular pseudosarcomatous fibroepithelial stromal polyp of the lower female genital tract 50 

B. Benign tumors and pseudotumors with reactive multinucleated, osteoclast-like giant cells 

• Suture granulomata 

• Textilomas 51 and teflonomas 52 

• Paraffinoma / oleogranuloma / silicone granuloma 



• Tumoral calcinosis 

• Tophaceous gout 

• Tophaceous pseudogout (nodular deposition of calcium pyrophosphate dihydrate crystals) 

• Dermatofibroma with osteoclast-like giant cells 

• Benign fibrous histiocytoma of subcutaneous and deep soft tissue 

• Fibroma of tendon sheath (variant) 

• Myofibroma / myofibromatosis (rare) 

• Fibromatosis (plantar type) 

• Giant cell collagenoma 

• Soft tissue aneurysmal bone cyst 

• Giant cell tumor of tendon sheath (localized-type) 53 

• Chondroma (calcified variant) 

• Giant cell tumor of the skin 

• Giant cell tumor of soft tissue 

• Giant cell tumor of mammary stroma 


• Florid reactive periostitis (fibroosseous pseudotumor of the digits) 

• Extraskeletal chondroma 

• Aggressive angiomyxoma (occasional) 

C. Borderline & malignant tumors with osteoclast-like giant cells 

• Atypical fibroxanthoma with osteoclast-like giant cells 

• Plexiform fibrohistiocytic tumor 

• Posphaturic mesenchymal tumor (mixed connective tissue variant) 

• Soft tissue giant cell tumor 

• Giant cell tumor of tendon sheath (diffuse type) 

• Leiomyosarcoma with osteoclastic-like giant cells 

• Malignant peripheral nerve sheath tumor with osteoclast-like giant cells 

• Extraskeletal osteoclastic(giant cel- rich) osteosarcoma 

• Osteoclastic giant cell malignant fibrous histiocytoma 

• Undifferentiated pleomorphic sarcoma with osteoclastic giant cells


• Osteoclastic-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts 53a 

• Malignant mesenchymoma with prominent osteoclastic giant cells 

• Metastatic osteoclastoma-like or giant cell-rich carcinoma 

D. Benign tumors with intrinsic multinucleated giant cells 

• Juvenile xanthogranuloma (Touton cells) 

• Multinucleated giant cells angiohistiocytoma of the skin 

• Kimura’s disease (polykaryocytes) 

• Langerhans’ cell histiocytosis 

• Myelolipoma (megakaryocytes) 

• Sclerosing extramedullary hematopoietic tumor (megakaryocytes) 

• Orbital and extra-orbital giant cell angiofibroma (relevant to solitary fibrous tumor group) 


• Giant cell angioblastoma of Nakagawa 

E. Borderline and malignant tumors with either intrinsic or accompanying neoplastic giant cells 


• Pleomorphic hyalinizing angiectatic tumor 

• Inflammatory myxohyaline tumor (inflammatory fibroblastic sarcoma) 

• Giant cell angiofibroma (of orbital and extra-orbital sites) 

• Malignant fibrous histiocytoma – storiform-pleomorphic type 

• Malignant fibrous histiocytoma – giant cell type 

• Myxoid malignant fibrous histiocytoma (gr. II) 

• Clear cell sarcoma 

• Alveolar rhabdmoyosarcoma with pleomorphic giant cells 

• Angiomatoid fibrous histiocytoma with pleomorphic giant cells 


• Alveolar soft part sarcoma 

• Soft tissue osteosarcoma 

• Malignant mesenchymoma 

• Melanoma (other than pleomorphic) 54 

• Epithelioid atypical angiomyolipoma 

• Any pleomorphic sarcoma (pleomorphic leiomyosarcoma, pleomorphic rhabdomyosarcoma, 

plemorphic liposarcoma, undifferendiated high grade pleomorphic sarcoma, and others) 

F. Malignant tumors with “malignant fibrous histiocytoma”-like pattern 


• Dermatofibrosarcoma protuberans with transformation to malignant fibrous histiocytoma 

• Dedifferentiated MFH-like areas in liposarcoma 

• Inflammatory and pleomorphic leiomyosarcoma 

• Malignant mesenchymoma with multinucleated osteoclastic-like cells 

• Malignant melanoma 55 

• Metastatic sarcomatoid/metaplastic carcinoma (e.g. renal cell carcinoma)

252 


Table VI. Tumors/pseudotumors with a distinctive architecture / growth pattern. 

A. Tumors and pseudotumors with fascicular pattern 

• Pseudoneoplastic leprosy (histoid variety) 

• Atypical mycobacterial spindle cell pseudotumor 

• Fibromatosis 

• Dermatomyofibroma 

• Myoid fibroma of the skin 

• Cellular schwannnoma 

• Leiomyoma (pilar, vascular type, ordinary) 

• Myoepithelioma (including storiform, herring-bone) 

• Dermatofibrosarcoma protuberans 

• Dermatofibrosarcoma protuberans with fibrosarcomatous areas (herring-bone) 

• Pigmented dermatofibrosarcoma protuberans (Bednar tumor) 

• Bednar tumor with fibrosarcomatous areas (herring-bone) 

• Fibrosarcoma (herring-bone) 

• Leiomyosarcoma 

• Myofibrosarcoma 56 

• Spindle cell liposarcoma 


• Fibrous monophasic synovial sarcoma 

• Fibrous synovial sarcoma (herring bone) 

• Leiomyomatous (spindle cell) rhabdomyosarcoma in children 

• Spindle cell rhabdomyosarcoma in adults 

• (Extra-)gastrointestinal stromal tumor 57 and other intra-abdominal spindle cell lesions 58 

• Follicular dendritic cell sarcoma 

• Cytokeratin-positive fibroblastic reticulum cell neoplasm 

• Melanoma 59 

B. Tumors with storiform (cartwheel) pattern 

• Benign fibrous histiocytoma 

• Neurofibroma 


• Cellular perineurioma (storiform perineurial fibroma) 

• Dermatofibrosarcoma protuberans (classic type) 


• Malignant fibrous histiocytoma (storiform-pleomorphic) 

• Metastatic sarcomatoid renal cell carcinoma 

C. Tumors and pseudotumors with whorled / curlicue pattern 

• Pacinian neuroma 

• Benign fibrous histiocytoma (epithelioid type with cellular whorls) (occasional) 

• Primary cutaneous and metastatic meningioma 

• Meningioma-like tumor of the skin 

• Nerve sheath tumor (benign, and malignant) 

• Nerve sheath myxoma (Pacinian neurofibroma) 

• Perineurioma (mainly cellular and Pacinian types) 

• Myopericytoma-type perivascular myoma 60 


• Spindle cell liposarcoma 

• Dedifferentiated liposarcoma (with meningothelial whorls) 


D. Tumors and pseudotumors with palisading pattern 

• Traumatic neuroma 

• Palisading cutaneous fibrous histiocytoma 

• Dermatofibroma with myofibroblastic differentiation 

• Palisaded angioleiomyoma 

• Benign nerve sheath tumor 

• Palisaded encapsulated neuroma / solitary circumscribed neuroma of the skin 


• Dermatofibrosarcoma protuberans (occasional)


• Primary cutaneous and metastatic meningioma 

• Neuroblastoma-like neurilemmoma 

• Leiomyoma 



• Fibrous synovial sarcoma (rare) 

• (Extra-)gastrointestinal stromal tumor 

• Malignant melanoma 

E. Tumors with amianthoid structures and (pseudo)rosettes 

• Dendritic cell neurofibroma with pseudorosettes 

• Myxopapillary ependymoma 

• Low-grade fibromyxoid sarcoma 

• Hyalinizing spindle cell tumor with giant rosettes 

• Intranodal palisaded myofibroblastoma 

• Soft tissue myofibroblastoma 


• Soft tissue perineurioma 

• Peripheral neuroepithelioma (PNET) / Ewing’s sarcoma 

• Neuroblastoma 

• Malignant peripheral nerve sheath tumor (rare) 

• Malignant lymphoma 

F. Tumors with cording / chordoid pattern 




• Mixed tumor 



• Extraskeletal myxoid chondrosarcoma, including the neuroendocrine variant 

• Round cell liposarcoma (rare) 

• Epithelioid variant of malignant peripheral nerve sheath tumor 

• Metastatic chordoma 



G. Tumors with endocrinoid pattern 

• Paraganglioma (zellballen) 

• Glomus tumor 

• Alveolar rhabdomyosarcoma, solid variant 

• Paraganglioma-like dermal melanocytic tumors 61 

• Metastatic renal cell carcinoma 


H. Tumors with alveolar pattern 

• Alveolar rhabdomyosarcoma 


• Ewing’s sarcoma (pseudoalveolar) 

• Metastatic renal cell carcinoma 

59, 59a 

• Melanoma 

I. Tumors with pseudo-glandular pattern 

• Adenomatoid tumor (lace-like or tubular pattern) 

• Pseudoglandular schwannoma 

• Tenosynovial giant cell tumor – diffuse type (cleft-like “pseudosynovial” spaces) 

• Monophasic epithelial synovial sarcoma 

• Monophasic epithelial and biphasic mesothelioma 

62, 62a 

• PNET with adamantinomatoid pattern 

• Melanoma 59

254 


J. Tumors with pseudo-vascular pattern 

• Hamartoma of the scalp with psedoangiosarcomatous features 

• Pseudoangiomatous hyperplasia of mammary stroma 


• Spindle cell lipoma – pseudoangiomatous variant 

• Angiomyofibroblastoma 


• Rhabdomyosarcoma –sclerosing, pseudovascular variant 


K. Tumors with retiform pattern 

i. Reticular – Netlike 

• Reticular perineurioma 

• Extraneurial retiform perineurioma 

• Hybrid perineurioma-schwannoma and perineurioma-neurofibroma tumors 


ii. Rete Testis-like 

• Retiform hemangioendothelioma 

• Composite hemangioendothelioma 

• Polymorphous hemangioendothelioma 

L. Tumors with (pseudo-)papillary pattern 

• Lipoma arborescens 

• Spindle cell lipoma (occasional) 

• Dabska’s tumor 

• Tenosynovial giant cell tumor – diffuse type 

• Extraspinal ependymoma (myxopapillary type) 


59, 59a 

• Melanoma


Table VII. Distinctive vascular patterned lesions (mainly non-endothelial). 

26 63 64 

A. Tumors with constant hemangiopericytoma-like pattern 

• Nasopharyngeal angiofibroma 

• Myofibroma and myofibromatosis 

• Myopericytoma . 

• Solitary fibrous tumor 


• Extraskeletal mesenchymal chondrosarcoma 

• Extrarenal renin-producing juxtaglomerular tumor 

• Spindle cell synovial sarcoma 

26 63 64 

B. Tumors with possible hemangiopericytoma-like pattern 

• (Pyogenic granuloma) 

• Hemangiopericytoma-like dermatofibroma 

• Epithelioid benign fibrous histiocytoma 

• Cellular fibrous histiocytoma 

• (Kaposi's sarcoma) 

• Infantile fibrosarcoma 

• Spindle cell lipoma with pseudoangiomaotus pattern 

• Malignant fibrous histiocytoma (storiform-pleomorphic) 


• Low grade fibromyxoid sarcoma (sometimes in recurrence) 


• Phosphaturic mesenchymal tumor 


• Liposarcoma (pleomorphic; dedifferentiated) 

• Glomus tumor (glomangiopericytoma) 


• Thymoma (predominantly spindle cell type / medullary type / type A) 

• Low grade endometrial stromal sarcoma - primary retroperitoneal; metastatic 

• Malignant mesothelioma – fibrous type 

• Metastatic sarcomatoid carcinoma (kidney, lung) 

• Melanoma – primary, metastatic 59 

C. Tumors with prominent perivascular hyalinization 

• Orbital & extraorbital giant cell angiofibroma 


• Cellular angiofibroma (external genital sites) 





• Extragastrointestinal gastrointestinal stromal tumor (not uncommon) 

D. Tumors with plexiform capillary pattern 

• Paraganglioma (mainly “branchiomeric type”) 

• Hemangioblastoma, extra-neuraxial 

• Myxoid liposarcoma 65 

• Myxoid malignant fibrous histiocytoma 

E. Tumors with glomeruloid capillary pattern 

• (Intravascular papillary endothelial hyperplasia - Masson’s phenomenon) 

• (Tufted angioma) 

• (Glomeruloid hemangioma) 

• (Dabska’s tumor) 66 

• Malignant angioendotheliomatosis (intravascular lymphoma) 

• Some neural and neuroendocrine neoplasms

256 


F. Lesions with hemorrhagic changes / pattern 

• (Organizing hematoma) 

• (Pyogenic granuloma) 

• (Kaposi’s sarcoma) 

• (Soft tissue Kaposiform hemangioendothelioma) 

• (Capillary hemangioma of the infantile hemangioendothelioma type) 

• Hemorrhagic dermatomyofibroma 

• Intranodal hemorrhagic spindle cell tumor with “amianthoid” fibres 

• Extraskeletal myxoid chondrosarcoma 


G. Tumors with aneurysmal changes / pattern 

• (Cavernous hemangioma, sinusoidal hemangioma, …) 

• Angiomatoid cellular blue nevus 66a 

• Aneurysmal bone cyst of soft tissue 

• Aneurysmal (“angiomatoid”) benign fibrous histiocytoma 

• Angiomatoid fibrous histiocytoma (angiomatoid “malignant fibrous histiocytoma”) 

• Atypical fibroxanthoma (aneurysmal or pigmented variant) 

• Dermatofibrosarcoma protuberans 67 

• Malignant fibrous histiocytoma giant-cell type 67 

• Soft tissue giant cell tumor 


• Cutaneous Ewing’s sarcoma 

• Paraganglioma (occasional) 

• Soft tissue telangiectatic osteosarcoma 

• Metastatic sarcomatoid carcinoma (mainly from kidney) 

• High grade sarcoma of various types (e.g. angiomatoid or angiectoid or angiosarcoma-like variant of epithelioid 

sarcoma 26a, 67a, 67b )


Table VIII. Combined, polyphasic, and heterologous tumors (other than collision 

tumors). 

A. Combined (homologous and heterologous) tumors 

• Combined dermatofibroma (two variants in the same lesion) 

• Giant cell fibroblastoma / dematofibrosarcoma protuberans 

• Giant cell fibroblastoma / Bednar tumor 

• Giant cell angiofibroma / dermatofibrosarcoma protuberans 

• Bednar tumor / dermal melanocytosis 

• Dermatofibrosarcoma protuberans / myxofibrosarcoma 

• Lipoma / leiomyoma 

• Angiomatosis / glomus cell proliferation 

• Acquired tufted angioma / vascular malformation 

• Lipoleiomyosarcoma 

• Neurofibroma / schwannoma 

• Neurofibroma / perineurioma 68 

• Schwannoma / perineurioma 69 

• Sarcomas (usually angiosarcomas) in malignant peripheral nerve sheath tumor 

• Mesenchymoma 

• Soft tissue ectomesenchymoma (benign and malignant Triton tumor, …) 

• Angiosarcoma in neurofibroma 

• Epithelioid malignancies in schwannomas 

59, 59a 

• Melanoma with adenoid pattern 

• Melanoma with neurosarcomatous transformation 

• Melanoma with ganglioneuroblastic differentiation 

• Rhabdomyosarcoma in a congenital melanocytic nevus 

B. Biphasic homologous (bimorphic) tumors 

• Lymphangiomyoma 

• Schwannoma (Antoni A & B areas). 

• Myofibroma and myofibromatosis 

• Fibrous hamartoma of infancy 

• Myolipoma (lipoleiomyoma) 

• Lipofibromatosis 

• Angiolipoma 

• Cellular angiolipoma 

• Angioleiomyoma 

• Neural fibrolipoma (fibrolipomatous hamartoma of nerve) 

• Lipomatous neurofibroma 

• Myelolipoma (adrenal; extraadrenal) 

• Fibrohistiocytic lipoma 

• Kaposi’s sarcoma 


• Dermatofibrosarcoma protuberans (with myoid differentiation; with nodular sclerotic changes) 

• Superficial / cutaneous angiomyxoma with epithelial component 

• Low grade fibromyxoid sarcoma 

• Hemosiderotic fibrohistiocytic lipomatous tumor 

• Intramuscular hemangioma with fatty component (can be viewed as triphasic) 

• Spindle cell lipoma in intramuscular lipoma (can be viewed as triphasic) 70 

• Angiomyolipoma (can be viewed as triphasic) 


• Mixed-type liposarcoma (dedifferentiated liposarcoma) 71 

• Low-grade dedifferentiated liposarcoma 72 

• Mixed-type liposarcoma (combined type: myxoid/round cell and pleomorphic liposarcoma) 

• Lipoleiomyosarcoma 

• Myxoid liposarcoma with nodules of round cell liposarcoma 

• Lipomatous hemangiopericytoma 


• Dedifferentiated parosteal osteosarcoma 

• Ganglioneuroma (mature Schwannian stroma and ganglion cells) 

• Ganglioneuroblastoma

258 


• Desmoplastic small round cell tumor (nests of undifferentiated tumor cells plus fibrous stroma) 

• Merkel cell tumor with squamous cell differentiation, and/or glandular differentiation 

C. Biphasic heterologous tumors – with epithelial elements 

• Hyaline cell rich-chondroid syringoma 

• Mixed tumors 

• Myoepitheliomas 


• Lipoma with synovial metaplasia 

• Ectopic hamartomatous thymoma 

• Superficial angiomyxomas / cutaneous myxomas 

• Schwannoma (with glandular differentiation; with squamous cell elements) 

• Malignant peripheral nerve sheath tumor with glandular and neuroendocrine differentiation; with squamous 

cell islands) 

• Ectomesenchymoma (benign, and malignant) 

• Malignant glandular Triton tumor 

• Biphasic synovial sarcoma with glandular differentiation 

• Epithelial synovial sarcoma with squamous cell differentiation 

• Pretibial soft tissue adamantinoma 

• Merkel cell tumor with leiomyo- or rhabdomyosarcomatous differentiation (see below at “E”) 

• Dermal squamomelanocytic tumor 73 

• Dermal basomelanocytic tumor 74 

D. Pseudo-biphasic tumors 

• Tumor growing in normal tissue 

i. Intramuscular lipoma 

ii. Intramuscular hemangioma 

iii. Intramuscular fasciitis 

iv. Proliferative myositis 

v. Fibromatosis colli 

vi. Intraneural leiomyoma 

vii. Intraneural glomus tumor 

viii. Intraneural synovial sarcoma 

ix. Sarcomas infiltrating fat 

• Tumor growing in tumor 

i. Schwannoma with metastatic carcinoma of the breast 

ii. Meningioma with metastatic carcinoma (various sources) 

iii. Malignant transformation in a benign tumor (e.g. angiosarcoma in hemangioma, Dabska tumor in a 

lymphangioma, malignant peripheral nerve sheath tumor in a ganglioneuroma, carcinoma arising in ectopic 

hamartomatous thymoma, carcinoma arising in a mixed tumor of soft tissue, …) 

E. Biphasic tumors with skeletal muscle differentiation 

• Rhabdomyomatous mesenchymal hamartoma of the skin 

• Leiomyoma and leiomyosarcoma 

• Dedifferentiated liposarcoma 


• Dedifferentiated chondrosarcoma 

• Neuromuscular choristoma (benign Triton tumor / benign ectomesenchymoma) 

• Malignant peripheral nerve sheath tumor / Malignant ectomesenchymoma (malignant Triton tumor) 

• Benign and malignant mesenchymoma 

• Ganglioneuroma and ganglioneuroblastoma 

• Merkel cell tumor 

• Metastasis from any tumors (of any lineage other than mesenchymal), possibly containing skeletal muscle 

component: germ cell tumors, blastomas, carcinosarcomas, tumors of specialized stroma [phyllodes tumors of 

breast, uterine adenosarcoma, Sertoli-Leydig cell tumor of the ovary], neuroendocrine carcinomas [Merkel 

cell tumor and other small cell carcinomas]


Table IX. Histogenetically diverse but morphologically similar tumors and 

pseudotumors with … 

A. Round-cell / epithelioid / plasmacytoid features 

• Epithelioid benign fibrous histiocytoma 


• Mixed tumor 

• Hyaline cell rich-chondroid syringoma 


• Epithelioid neurofibroma 

• Epithelioid schwannoma 

• Cutaneous epithelioid Schwannoma 75 

• Epithelioid hemangioma 

• Glomus tumor 

• Epithelioid leiomyoma 

• Epithelioid angioleiomyoma 

• Tenosynovial giant cell tumor – diffuse type 






• Epithelioid sarcoma-like hemangioendothelioma 

• Epithelioid leiomyosarcoma 

• Epithelioid variant of malignant peripheral nerve sheath tumor 

• Epithelioid angioleiomyoma 

• Epithelioid angiomyolipoma and other PEComas 76-78 

• Epithelioid angiosarcoma 

• Poorly differentiated fibrosarcoma 


• Round cell liposarcoma 

• Epithelioid liposarcoma 

• Extraskeletal myxoid chondrosarcoma – epithelioid variant 


• Proximal-type epithelioid sarcoma 

• Extrarenal malignant rhabdoid tumor 

• Biphasic & monophasic epithelial synovial sarcoma 

• Biphasic & monophasic epithelial mesothelioma 

• Deciduoid mesothelioma 79-81 

• Extranodal (epithelioid) histiocytic sarcoma 82 

B. Clear cell changes 83 

• Fibrous papule of the nose, clear cell variant 84 

• Clear cell dermatofibroma 

• Myoepithelioma – clear cell type 

• Angioleiomyoma – clear cell variant 

• Angiomyolipoma 

• Leiomyoma – clear cell variant 

• Atypical fibroxanthoma - clear cell type 

• PEComas of soft tissue 76 

• Abdominopelvic PEComa 77 

• PEComas of the skin 78 

• Distinctive dermal clear cell mesenchymal neoplasm 85 

• Epithelioid leiomyosarcoma 

• Epithelioid malignant peripheral nerve sheath tumor 


• Alveolar rhabdomyosarcoma – clear cell variant 

• Ewing’s sarcoma – clear cell variant 85a 


• Ectopic hamartomatous thymoma with cords of clear cells 

• Alveolar soft part sarcoma (clear cell degenerative variant) 

• Balloon cell nevus 

• Balloon cell blue nevus 

• Balloon cell melanoma 

• Paraganglioma-like dermal melanocytic tumor 

• Metastasis of clear cell tumors from other organs 

• Peripheral T-cell lymphoma “unspecified”

260 


C. (Uni-/multi-)vacuolated (pseudo-)lipoblasts, and (pseudo-)physalipherous cells 

• Silicone granuloma 


• Atrophic fat in malnutrition 

• Polyvinylpyrrolidone pseudotumoral histiocytic reaction (“mucicarminophilic histiocytosis”) 

• Hibernoma 

• Rhabdomyoma (secondary vacuoles to loss of glycogen) 


• Pseudolipoma (battered buttock syndrome) 

• Spindle cell hemangioma 



• Adenomatoid tumor (pseudovacuolated cells) 


• Inflammatory myxohyaline tumor 

• Low grade myxofibrosarcoma (myxoid fibrosarcoma or myxoid malignant fibrous histiocytoma gr. I) 

• Myxoid malignant fibrous histiocytoma (gr. II) 

• Liposarcoma 

• Pseudolipoblastic (“signet-ring”) nerve sheath tumor 85b 

• Lipoblastic meningioma 

• Chordoma - metastatic, primary heterotopic 28 28b , periphericum 32a 

• Ewing’s sarcoma 

• (Extra-)gastrointestinal stromal tumor 85c 

• Large cell lymphoma (signet ring cell type) 

59a, 85d-e 

• Melanoma (signet ring-cell type - primary, and metastatic) 

• Metastatic adenocarcinoma 

86 87 

D. Eosinophilic / oncocytic features 

• Silica reaction 

• Crystal storing histiocytosis 

• Rhabdomyoma – oncocytic type 

• Glomus tumor – oncocytic type 

• Paraganglioma – oncocytic type 

• Oncocytic myoepithelioma 

• Soft tissue oncocytoma 

• Extraskeletal myxoid chondrosarcoma (cellular variant) 


• Malignant extrarenal rhabdoid tumor 


• Some benign and malignant melanocytic tumors 

E. Granular cells 

• Granular fibrous papule of the face 21 

• Granular histiocytic cell reaction 

• Granular stromal cell reaction with various tumors 

• Granular cell tumor / granular cell schwannoma 

• Congenital gingival granular cell tumor 

• Granular cell dermatofibroma 

• Atypical fibroxanthoma with granular cells 

• Dermatofibrosarcoma protuberans with granular cells 

• Primitive polypoid granular cell tumor (of the skin) 

• Primitive nonneural granular cell tumors of skin (the same as the previous one) 88 

• Granular cell perineurioma 

• Granular cell leiomyoma 

• Hibernoma 

• Malignant granular cell tumor 

• Granular cell leiomyosarcoma 

• Granular cell angiosarcoma 

• Some benign and malignant melanocytic tumors 

F. Rhabdoid- / pseudo-rhabdoid features 

• Crystal-storing histiocytosis 

• Adult rhabdomyoma 

• Rhabdomyomatous mesenchymal hamartoma 

• Myoepithelioma


• Leiomyosarcoma with rhabdoid features 

• Rhabdomyosarcoma with rhabdoid features 


• Extraskeletal myxoid chondrosarcoma – rhabdoid variant 

• Epithelioid malignant peripheral nerve sheath tumor 





• [Also carcinoma, lymphoma, meningioma, and primary and metastatic melanoma 59a, 85c-d can acquire rhabdoid phe 

notype] 

G. Ganglion / ganglion-like cells 

• Ischemic fasciitis (Atypical decubital fibroplasia) 

• Ganglion cell choristoma of skin 

• Cutaneous ganglion cell tumor 

• Cutaneous ganglioneuroma 

• Desmoplastic cutaneous ganglioneuroma 

• Proliferative fasciitis 

• Proliferative myositis 

• Ganglioneuroma 

• Dendritic cell neurofibroma 

• Paraganglioma-like dermal melanocytic tumor 

• Ectomesenchymoma 

• Differentiating neuroblastoma & ganglioneuroblastoma 

H. Small round cell tumors 89 

• Glomus tumors (benign, borderline, malignant) 

• Tenosynovial giant cell tumor 

• Primitive neuroectodermal tumor / extraskeletal Ewing’s sarcoma 

• Malignant hemangiopericytoma 

• Round cell liposarcoma 


• Neuroblastoma 



• Embryonal and alveolar rhabdomyosarcoma 


• Extraskeletal myxoid chondrosarcoma – cellular variant 

• Small cell osteosarcoma 

• Malignant lymphoma 

• Granulocytic sarcoma / leukaemia 

• Extramedullary myeloid cell tumors 

59a, 85d 


• Small cell carcinoma (primary: Merkel cell-type, and non-Merkel cell type; metastatic: pulmonary, and 

extrapulmonary) 

I. Pleomorphic soft tissue tumors 

• Benign or intermediate 

i. Pleomorphic fibrous papule of the face 21 

ii. Pleomorphic fibroma of the skin 

iii. Subungual pleomorphic fibroma 

iv. Subungual myxoid pleomorphic fibroma 

v. Pleomorphic hyalinizing angiectatic tumor 

vi. Hemosiderotic fibrohistiocytic lipomatous tumor 

vii. Atypical fibroxanthoma 

• Malignant 90 

viii. Storiform-pleomorphic malignant fibrous histiocytoma 

ix. Dedifferentiated areas in well-differentiated liposarcoma 

x. Pleomorphic liposarcoma 91 

xi. Pleomorphic leiomyosarcoma 92 

xii. Pleomorphic myofibrosarcoma 

xiii. Pleomorphic rhabdomyosarcoma 

xiv. Pleomorphic malignant peripheral nerve sheath tumor 

xv. Pleomorphic intimal sarcoma 93 

xvi. Malignant PEC-oma (may mimic pleomorphic myogenic sarcoma)

262 


Table X. Tumors and pseudotumors with diagnostic clues. 

A. Architecturally distinct features 

• Swiss cheese pattern (oleogranuloma / paraffinoma, silicone granuloma, fat necrosis, chalazion, …) 

• Verocay bodies and Verocay-like bodies or nuclear palisading (benign and malignant nerve sheath tumors, 

leiomyoma, leiomyosarcoma, spindle cell synovial sarcoma, extragastrointestinal gastrointestinal stromal tumors) 

• Homer-Wright rosettes (neuroblastoma, Ewing’s sarcoma, PNET, Merkel cell carcinoma) and perivascular 

pseudorosettes (ependymoma) 

• Amianthoid fibers, or giant collagen rosettes (palisaded myofibroblastoma, hyalinizing spindle cell 

tumor with giant rosettes, dendritic cell neurofibroma with pseudorosettes and other nerve sheath 

tumors, solitary fibrous tumor, osteocartilaginous tumors, others) 

• Nonspecific rosettes (non-Hodgkin lymphoma – occasional; epithelioid osteosarcoma, melanoma) 

• “Zellballen” (paraganglioma) 

• Cambium layer (botryoid embryonal rhabdomyosarcoma) 

• Filigree pattern (Ewing’s sarcoma) 

• Light cell/dark cell phenomenon – marble appearance (Ewing’s sarcoma, MPNST) 

• Azzopardi’s effect (Merkel cell tumor; other small cell tumors) 

• Zoning phenomenon from fibroblastic center to ossified periphery (myositis ossificans) 

• Reverse zoning phenomenon (osteosarcoma) 

• Pacinian corpuscle (Pacinian neuroma) 

• Wagner-Meissner body (diffuse neurofibroma) 

• Pacinian body-like and Meissner body-like structures (nerve sheath tumors) 

• Onion-bulb structures (hereditary diffuse hypertrophic neuropathies: Charcot-Marie-Tooth disease, Dejerine-Sottas 

disease; other true onion bulb neuropathies: Refsum disease, Krabbe’s disease, Roussy-Levi disease, metachromatic 

leukodystrophy, neurofibromatosis type 1, diabetic neuropathy, chronic inflammatory demyelinating 

polyradiculoneuropathy; localized hypertrophic inflammatory neuropathy; “localized hypertrophic neuropathy” 

as true onion bulb neuropathy) 

• Pseudo-onion bulb structures (“localized hypertrophic neuropathy” as intraneural perineurioma; 

extraneural/soft tissue perineurioma) 

• Cytophagocytosis (cytophagic histiocytic panniculitis/subcutaneous panniculitis-like T-cell lymphoma; inflammatory 

malignant fibrous histiocytoma; alveolar rhabdomyosarcoma, melanoma, …) 

• Emperipolesis (Rosai-Dorfman disease, inflammatory myxohyaline tumor 94 , inflammatory liposarcoma 95 ) 

B. Extracellular findings / stromal precipitates 

• Schaumann bodies (sarcoid, sarcoid-like granulomatous reactions) 

• (Pseudo-)psammomatous calcifications (calcifying fibrous pseudotumor) 

• Psammoma bodies (cutaneous meningioma) 

• Congophilic green birefringent material (amyloid tumor) 

47, 48 

• Non-congophilic amorphous material in pelvic and retroperitoneal soft tissue (Tamm-Horsfall protein deposition) 

• Non-congophilic positively birefringent microcrystals (tumoral pseudogout) 

• Non-congophilic, needle-shaped birefringent crystals (tophaceous gout) 

• Foreign bodies (even partly intracellular) (silica [soil, glass], talc, starch, condom emulsion, silicone, cactus spine, seaurchin 

spine, hairs, wooden splinters, suture material, dacron graft, woollen swab, …) (foreign body reactions 

and granulomas, pseudotumoral granulomatous reactions, textilomas, …) 

• Non-congophilic amorphous material in pelvic and retroperitoneal soft tissue (Tamm-Horsfall protein deposition) 47,48 

• Petaloid globules – chenille bodies (elastofibroma) 

• Skeinoid fibres (intestine-based stromal tumor with neural differentiation, and rarely extra-gastrointestinal GIST) 

• Ferruginous bodies – asbestos bodies (mesothelioma) 

• Intravascular fibrin thrombi (-angiolipoma) 

C. Cytohistologically distinct features 

• Cell peculiarities 

– Fleurette-like cells (pleomorphic lipoma, giant cell angiofibroma) 

– Touton cells (juvenile xanthogranuloma, Erdheim-Chester disease) 

– Reed-Sternberg-like cells (inflammatory myxo-hyaline tumor, proliferative myositis, some extranodal soft 

tissue large cell lymphomas, …) 

– Spider cells (rhabdomyoma) 

– Tombstone-cells (epithelioid hemangioma) 

– Hobnail-cells (hob-nail hemangiomas – Dabska’s type, retiform; polymorphous hemangioendothelioma, …) 

– Vacuolated cells (lipoblast → liposarcoma; lipoblast-like stromal cells → myxofibrosarcoma; lipoblast-like 

macrophage → fat necrosis, oleogranulomas; bubbly histiocytes → polyvynilpyrrolidone reaction; 

endothelioblast and endotheliocyte → vascular tumors; …)


– Strap cells / cross-striated cells (rhabdo-myomatous/-myosarcomatous tumors) 

– Longitudinally pseudostriated cells (crystal storing histiocytosis in lymphoproliferative disorders) 

• Intracellular findings 

i. Melanin (melanocytic tumor; neural tumor; neuroectodermal tumor) 

ii. Intracytoplasmic hyaline globules (Kaposi’s sarcoma, glomeruloid hemangioma, Kaposiform 

hemangioendothelioma) 

iii. Trichrome stain positive intracytoplasmic inclusions (infantile digital fibromatosis; myofibrosarcoma) 

iv. Granular material in reactive histiocytes (site of previous surgery) 

v. Intracytoplasmic small black particles in foamy histiocytes (prosthetic heavy metal detritus) 

vi. Intracytoplasmic PAS-D resistant bodies (granular cell tumor; extraintestinal Whipple’s disease 96 97 ) 

vii. Intracellular crystalline PAS-D positive material (alveolar soft part sarcoma) 

viii.Intracytoplasmic paracrystalline material (crystal storing histiocytosis in lymphoproliferative disorders) 

ix. Michaelis-Gutmann bodies (malakoplakia) 

x. Asteroid bodies (sarcoid, sarcoid-like granulomatous reactions, …) 

xi. Infectious bacilli (bacillary angiomatosis, mycobacterial disease, atypical mycobacterial disease, Whipple disease) 

xii. Cytoplasmic cross-striations (rhabdo-myomatous / -myosarcomatous tumors) 

xiii.Cytoplasmic fibrillary pattern (“wrinkled cigarette paper” in Gaucher disease, …) 

xiv. Cytoplasmic intranuclear pseudoinclusions (meningioma, nerve sheath tumors, melanocytic tumors, soft tissue 

lymphoplasmacytic proliferative disorders [Dutcher bodies], metastatic hepatocellular carcinoma) 

xv. Nuclear vacuoles – lochkern (normal fat) 

xvi.Nuclear grooves (soft tissue metastatic thyroid papillary carcinoma; metastatic granulosa cell tumor; melanoma)

264 


Table XI. Histologically* pigmented tumors and pseudotumors with … 

A. Melanin pigment 

• Fibrous papule of face –pigmented variant 21 

• Cutaneous benign fibrous histiocytoma (clinically pigmented) 

• Intradermal Spitz nevus 

• Dermal dendritic melanocytic proliferations 98 (including deep seated blue nevus) 

99 100 

• Pigmented pilomatrixoma and melanocytic pilomatrixoma 

• Pigmented (melanotic) neurofibroma 

• Pigmented (melanotic) schwannoma 

• Psammomatous melanotic schwannoma 

• Cutaneous psammomatous melanotic schwannoma 101 


• Bednar tumor associated with dermal melanocytosis 

• Neurocristic cutaneous hamartoma 

• Neurocutaneous melanosis (melanoma, melanoblastoma, meningeal melanocytosis) 

• Pigmented (melanotic) neuroectodermal tumor of infancy 

• Ordinary melanoma 

• Primary dermal melanoma 102 

• Desmoplastic melanoma 

• Deep melanoma 

• Metastatic melanoma 

• Pigmented epithelioid melanocytoma 103 / animal-equine-type melanoma 104 / epithelioid blue nevus (sporadic, 

and syndromic – associated with Carney’s syndrome 105 ) 


• Pigmented ganglioneuroblastoma 

• Phaeochromocytoma and other extraadrenal paraganglioma 106 

• Deep melanoma 

• [Melaning-producing non-mesenchymal tumors (pigmented carcinoid, thyroid medullary carcinoma)] 

B. Hemosiderin 

• Hemorrhage / hematoma / ancient hematoma 

• Hemangiomas (mainly cavernous type) and phakomatoses 

• Synovial hemangioma 

• Hemosiderotic dermatofibroma 

• Targetoid hemosiderotic hemangioma 

• Aneurysmal benign fibrous histiocytoma 

• Aneurysmal (pigmented) atypical fibroxanthoma 



• Hemosiderotic fibrohistiocytic lipomatous tumor 

• Diffuse-type giant cell tumor (pigmented villonodular synovitis / pigmented villonodular bursitis) 


• Tenosynovial giant cell tumor (localized and diffuse type) 


C. Purified carbon / charcoal 

• Preoperative localization of gut biopsy sites 

• Preoperative localization of nonpalpable tumor lesions 

D. India Ink (rare), Cinnabar, Chrome green, Cobalt blue 

• Delayed hypersensitivity reactions to tattoos 

E. Heavy metals particles 

• Paraarticular histiocytic proliferation induced by cobalt-chromium and titanium with intracytoplasmic small black 

particles in histiocytes (prosthetic heavy metal detritus) 

[* Clinically pigmented or erythematous tumors and pseudotumors without pigment at histology: Aschoff's nodules (rheumatic fever), Farber's lipogranulomatosis, Sweet's 

syndrome, panniculitis, juvenile xanthogranuloma, mastocytoma, cutaneous T-cell lymphoma, metastatic cutaneous neuroblastoma]


Table XII. Clinically alarming benign tumors and pseudotumors. 

A. Multiple / multicentric, possibly recurring benign lesions 

• Cutaneous sarcoid 

• Papular angiokeratoma (a frequent mimicker of melanoma) 

• Multiple eruptive angiofibromas 

• Multiple eruptive dermatofibromas (in HIV and immunosuppressed patients) 

• Recurrent pyogenic granuloma with satellitosis 107 

• Multiple pyogenic granulomas 

• Generalized pyogenic granuloma 

• Multiple nevus lipomatosus cutaneous superficialis 

• Multiple cutaneous pilar leiomyomas 

• Multiple cutaneous chondromas 

• Multiple miliary osteomas of the face 

• Nodular fasciitis (satellite nodules) 

• Infantile fibromatosis (desmoid-type) 

• Juvenile hyaline fibromatosis 22 

• Juvenile lipofibromatosis 

• Infantile digital fibromatosis-recurring 

• Multiple juvenile xanthogranuloma 

• Multicentric reticulohistiocytosis / multiple reticulohistiocytic granulomata / progressive nodular histiocytoma / 

generalized eruptive histiocytoma, xanthoma disseminatum 

• Tendinous xanthomas 

• Xanthogranulomatous inflammation (e.g. in Erdheim-Chester disease) 

• (Recurring) tumoral calcinosis 

• (Recurring) tophaceous pseudogout (CPPD crystal deposition disease - tumoral form) 

• Infantile myofibromatosis 

• Multiple multifocal (adult) rhabdomyomas of the neck 108 

• Multiple intramuscular myxomas 

• Myolipoma (of soft tissue) 

• Inflammatory pseudotumor / inflammatory myofibroblastic tumor 

• Infantile hemangiopericytoma 

• Multiple glomus tumors 

• Glomangiomatosis (may recur) 

• Multiple glomangiomyomas 

• Infiltrating (aggressive, atypical) glomus tumor 

• Recurring glomus tumor 

• Plexiform neurofibroma 

• Multiple schwannomas (schwannomatosis) 

• Multiple cutaneous plexiform schwannomas 

• Multiple melanotic schwannomas 

• Multiple cellular neurothekeomas 109 

• Giant cell fibroblastoma (recurring) 

• Multiple lipomas 

• Multiple spindle cell lipomas (familial and non-familial forms) 

• Multiple angiolipomas (familial and non-familial forms) 

• Symmetric lipomatosis 

• Diffuse lipomatosis 

• Diffuse angiomatosis 

• Multiple spindle cell hemangio(endothelio)mas 

• Spindle cell hemangiomatosis 

• Fibrodysplasia (myositis) ossificans progressiva 

• (Recurring) bizarre parosteal osteochondromatous proliferation (Nora’s disease) 

• Multiple (multifocal) leiomyomas 

• Leiomyomatosis peritonealis disseminata 

• Granular cell tumor 

• Progressive lymphangioma (benign lymphangioendothelioma) 

• Reactive angioendotheliomatosis 

• (Epithelioid hemangioendothelioma) 


• Cerebriform fibrous proliferations in Proteus syndrome

266 


• (Some) multiple smooth muscle tumors in immunocompromised patients 

• Paraganglioma (multicentric) 

24, 25 


43, 44 

• Sclerosing mesenteritis, mesenteric panniculitis, mesenteric lipodystrophy, and other inflammatory pseudotumors 

• Multiple fibrosclerosis (any combination of Ormond’s disease, sclerosing cholangitis, mediastinal fibrosis, Riedel’s 

thyroiditis, pseudotumor of th orbit, autoimmune pancreatitis) 

• Sclerosing mediastinitis (idiopathic fibroinflammatory lesions of the mediastinum) 25a 

B. Painful soft tissue tumors and pseudotumors 

• Traumatic neuroma 

• Keloid (sometimes) 

• Dercum’s disease (adiposis dolorosa) 

• Glomus tumors (see section A, above) 

• Angiolipoma 

• Angioleiomyoma 

• Cutaneous leiomyoma 

• Morton’s interdigital neuroma 

• Schwannomatosis


Table XIII. Pathologically alarming benign tumors and pseudotumors. 

A. Lesions with multilobular / mosaic growth pattern 

• Tumoral calcinosis 

• Lipoblastoma 

• Cutaneous myxoma 


• Juvenile hemangioma 

• Pyogenic granuloma 

• Acquired tufted angioma 


• Extraskeletal chondroma 

• Synovial chondromatosis (articular and extra-articular variant) 

• Giant cell tumor 

• Intramuscular myxoma (occasional) 

• Ossifying fibromyxoid tumor (not including here the atypical & malignant variant) 

B. Lesions with plexiform / infiltrating margins 


• Cranial fasciitis 



• Fibromatosis (any type, mainly infantile type) 

• Plexiform xanthomatous tumor 

• Plexiform Schwannoma (cutaneous and subcutaneous, and deep forms) 109a / Schwannomatosis 109b-d 


• Neurothekeoma (classic and cellular type) 

• Nerve sheath myxoma (“plexiform myxoma”) 

• Granular cell tumor (plexiform variant) 

• Dendritic fibromyxolipoma 

• Intramuscular lipoma (infiltrating lipoma) 

• Diffuse lipoblastomatosis (infiltrating lipoblastoma) 

• Lipoma arborescens 

• Cellular schwannoma (occasional) 

• Congenital and childhood plexiform (multinodular) cellular schwannoma 110 

• Deep-seated plexiform schwannoma 111 

• Infiltrating retiform perineurioma 112 


C. Lesions with intravascular growth 

• [Intralymphatic granulomas (in Crohn’s disease, granulomatous lymphangitis of the scrotum and penis, 

granulomatous cheilitis, Melkerson and Rosentahl syndrome)] 

• Organizing thrombus 

• Pyogenic granuloma – intravascular variant 



• Plasma cell granuloma 


• Intravenous atypical vascular proliferation 

• Intravascular papillary endothelial hyperplasia 

• Spindle cell hemangioma 


• Myopericytoma – intravascular variant 

• Myofibroma (solitary type – adult form) 



• Intravascular glomus tumor 

• Intravascular fasciitis 

• Angioleiomyoma – intravascular form 

• Intravenous leiomyomatosis (conventional and unusual variants) 113 

• Myointimoma 

• Tenosynovial giant cell tumor

268 


D. Benign lesions with “hypercellularity” 

• Cellular fibrous papule of the face 21 

• Cellular digital fibromas 

• Cellular dermatofibroma 

• Cellular benign fibrous histiocytoma 

• Cellular pseudosarcomatous fibroepithelial stromal polyp 

• Cellular infantile capillary hemangioma 114 

• Cellular spindle cell hemangio(endothelio)ma 

• Cellular angiolipoma 

• Cellular schwannoma 115 

• Cellular schwannoma with granular cells 

• Congenital and childhood plexiform (multinodular) cellular schwannoma 110 

• Cellular neurofibroma 

• Cellular leiomyoma 

• Cellular perineurioma 

• Cellular neurothekeoma 

• Infantile fibromatosis (desmoid-type) 

• Cellular fibromatosis (palmar type) 

• Monophasic cellular variants of myofibromatosis 116 

• Cellular giant cell fibroblastoma 

• Cellular intramuscular myxoma 

• Cellular rhabdomyoma of fetal type (intermediate variant) 

• Cellular tenosynovial giant cell tumor 

• Cellular ossifying fibromyxoid tumor 

• Cellular blue nevus


Table XIV. Histologically benign lesions (other than vascular proliferations) mistaken 

for malignant. 


• Subcutaneous granuloma annulare (mimicking epithelioid sarcoma, epithelioid angiosarcoma) 

• Mitotic granuloma annulare 

• Necrobiotic xanthogranuloma 


• Nodular fasciitis and variants (intravascular, cranial, peri- and parosteal, intraarticular fasciitis) 

• Ischemic fasciitis (atypical decubital fibroplasia) 



• Desmoid fibromatosis 

• Reticulohistiocytoma 

• (Juvenile) xanthogranuloma with inconspicuous foam cells and giant cells (mimicking melanoma) 

• Polyvynilpyrrolidone pseudotumoral histiocytic reaction (“mucicarminophilic histiocytosis”) 

simulating signet-ring cell carcinoma, and myxoid liposarcoma 

• Tumefactive fibroinflammatory lesion 

• Xanthogranulomatous pseudotumor (several etiologies, mainly infectious) 

• Epithelial sheath neuroma 

• Reactive angioendotehliomatosis 

• Intramuscular hypertrophic scar following sarcoma resection 

• Massive localized lymphedema in morbid obesity 

• Pseudoneoplastic skeletal muscle regeneration 

• Post-implant pseudoneoplastic parathyromatosis 

• Hyperplastic callus in pathologic conditions (e.g. osteogenesis imperfecta) 

• Tumefactive soft tissue extension of Paget’s pseudosarcoma 

• Tophaceous pseudogout 

• Myositis-, panniculitis-, fasciitis- and periostitis ossificans 

• Fibrodysplasia ossificans progressiva 

• Fibroosseous pseudotumor of the digits 

• Bizarre parosteal osteochondromatous proliferation (Nora's lesion) 

• Vulvar hypertrophy with lymphedema 

• Cutaneous pseudosarcomatous polyp 

• Vaginal pseudosarcomatous fibroepithelial polyp 50 

• Pseudotumoral deciduosis 

• Extramedullary hematopoiesis (e.g. in breast, in retroorbital site, …) 

• Sclerosing extramedullary hematopoietic tumor 


• Granular cell dermatofibroma 

• Benign cellular fibrous histiocytoma 

• Atypical (pseudosarcomatous) fibrous histiocytoma of the skin 

• Atypical cellular neurothekeoma 

• Deeply located benign fibrous histiocytoma 

• Intraneural glomus tumor 

• Symplastic glomus tumor 

• Intraneural leiomyoma 


• Symplastic leiomyoma 


• Plexiform (multinodular) cellular schwannoma 110 

• Intramuscular hemangioma 

• Synovial chondromatosis (extra-articular variant) 

• Aggressive angiomyxoma

270 


Table XV. Reactive endothelial lesions that may histologically mimick malignancy. 

• Organizing hematoma 

• Ancient hematoma 

• Intravascular papillary endothelial hyperplasia 

• Acroangiodermatitis 

• Glomeruloid hemangioma 


• Acquired progressive lymphangioma / benign lymphangioendothelioma 

• Reactive angioendotheliomatosis 

• Benign atypical vascular lesions of the lip 

• Multiple trauma-induced targetoid hemosiderotic hemangioma-like lesions 

• Diffuse dermal angiomatosis of the breast (response to isotretinoin therapy) 

• Acquired vulvar lymphangioma (secondary to Crohn’s disease) 

• Pyogenic granuloma-like lesion associated with topical retinoid therapy 

• Pseudokaposiform reaction to Monsel’s solution 

• Florid vascular proliferation as a needle biopsy effect in the breast 

• Post-radiation vascular proliferations 117 

Table XVI. Histologically malignant tumors mistaken for benign 118 . 

• Inflammatory mesenchymal sarcomas (“pseudo-pseudotumors”) (e.g. inflammatory leiomyosarcoma, inflammatory 

fibrosarcoma, inflammatory malignant fibrous histiocytoma) 

versus myofibroblastic pseudotumors and tumefactive fibroinflammatory lesions 

• Low grade fibromyxoid sarcoma 

versus myxoid neurofibroma, perineurioma, desmoid fibromatosis, nodular fasciitis 

• Low grade myxofibrosarcoma 

versus myxoid neurofibroma, perineurioma, intramuscular / juxta-articular myxoma, superficial angiomyxoma 

• Low grade myofibroblastic sarcoma 

versus desmoid fibromatosis 

• Well differentiated lipoma-like liposarcoma 119 

versus ordinary lipoma, hibernoma 

• Well differentiated sclerosing type liposarcoma 

versus pleomorphic lipoma 

• Well differentiated inflammatory type liposarcoma 120 

versus inflammatory process 

• Well differentiated spindle cell (neural-like) liposarcoma 121 

versus benign peripheral nerve sheath tumor 


versus pseudogranulomatous inflammatory process (deep granuloma annulare, rheumatoid nodule, deep 

rheumatoid nodule, necrobiosis lipoidica, necrobiotic granuloma, fibrous benign tumors 67b ) 

• Subcutaneous panniculitis-like T cell lymphoma & granulomatous slack skin 

versus lupus profundus vs lymphocytic lobular panniculitis vs. atypical lymphocytic lobular panniculitis 

• Histiocytic sarcoma 

versus benign histiocytosis 

• Non-mesenchymal tumors in special locations (low grade sarcomatoid carcinoma in the breast) 

versus fibromatosis 122


Table XVII. Atypical variants of typical tumors. 

A. By clinical features (the so-called “man from Istanbul” 123 ) 

• Any typical superficial soft tissue tumor in deep location (e.g. deep juvenile xanthogranuloma – subcutaneous, 

intramuscular, intraneural and visceral forms 124 125 ; retroperitoneal spindle cell lipoma; retroperitoneal 

Pacinian neuroma; soft tissue deep and visceral 126 127 benign fibrous histiocytoma; deep - subcutaneous 

dermatofibrosarcoma protuberans without dermal involvement; …) 

• Any typical deep soft tissue tumor in very superficial location (e.g. cutaneous rhabdomyosarcoma; cutaneous – 

intradermal liposarcoma; Ewing’s sarcoma of the skin; adamantinoma of the pretibial soft tissue; …) 

• Any typical tumor in heterotopic/unusual locations (e.g. fibrous hamartoma of infancy in hands or feet; 

non-midline / non-paraxial paraganglioma; subcutaneous/intravascular/visceral-organ based pyogenic granuloma, …) 

• Kimura's disease in non-Orientals 

• “Extra-nodal” Castleman’s disease 

• “Extra-nodal” Rosai-Dorfman disease 

• “Extra-nodal” dendritic cell and interdigitating cell sarcomas 

• “Extra-abdominal” intra-abdominal desmoplastic round cell tumor (pleura, meninges, …) 

• “Extra-orbital” (orbital) giant cell angiofibroma 

128, 128a 

• “Extra-nasopharyngeal” nasopharyngeal angiofibroma 

• “Extra-renal” renin-producing juxtaglomerular tumor 

• “Extra-renal” rhabdoid tumor (e.g., soft tissue, skin, vulva, …) 

• “Non-acral” acral inflammatory myxohyaline tumor 

• “Extra-acral” (acral) calcifying aponeurotic fibroma 

• “Non-digital” (long bones) fibroosseus pseudotumors of the digits 

• “Extra-acral” (long bones, skull, maxilla) bizarre osteochondromatous proliferation of the small tubular 

bones of the hands and feet 

• “Extra-inguinal” intranodal palisaded myofibroblastoma 

• “Non-ossifying” ossifying fibromyxoid tumor 

• “Extra-osseus” aneurysmal bone cyst 

• “Extra-osseous” adamantinoma of the pretibial soft tissue 

• “Extra-articular” synovial sarcoma (e.g., primary cutaneous, intraabdominal 129 , ...) 

• “Extra-articular” (articular) pigmented villonodular synovitis / extraarticular diffuse type giant cell tumor 

• “Ectopic” ectopic hamartomatous thymoma 

• “Extra-genital” (genital/paragenital) calcifying fibrous pseudotumor 

• “Extra-gastrointestinal” gastrointestinal stromal tumor 

• “Extra-digital” infantile digital fibromatosis 

• “Extra-mammary” mammary-type myofibroblastoma 

• “Extra-neuraxial” cerebellar-type hemangioblastoma 

• “Extra-uterine” (parasitic) uterine leiomyoma 

• “Extra-vulval” (vulval) cellular angiofibroma 

• (Vulval) angiomyofibroblastoma in males (scrotum) 

• (Vulval) angiomyofibroblastoma-like tumor in males (analogous to cellular angiofibroma) 

• Aggressive angiomyxoma (of the female pelvis) in males 

• Nasopharyngeal angiofibroma (of the males) in females 

• Juvenile xanthogranuloma in adults 

• Infantile digital fibromatosis in adults 

• Infantile myofibromatosis in adults 

• Calcifying fibrous pseudotumor of childhood in adults 

• Kaposiform (infantile) hemangioendothelioma in adults 

• Non-infantile (infantile) nasal chondromesenchymal hamartoma 130 

• Ectopic meningioma 

• Pediatric-type sarcomas in adults (including embryonal & botryoid rhabdomyosarcoma) PNET/EWS, angiomatoid 

malignant fibrous histiocytoma, plexiform fibrohistiocytic tumor) 131 

• Spindle cell rhabdomyosarcoma in adults 132 

• Liposarcoma in children (questioned – debated) 

• MPNST in children (questioned – debated) 

• “Extra-medullary” myeloid tumors (lymph node, upper respiratory tract, female genital tract, soft tissue, body 

cavities …) 

• “Extraosseous” plasmacytoma (upper respiratory tract, lymph node, soft tissue, skin, mediastinum, …) 

• “Extraneuraxial” (retroperitoneal malignant) meningioma 133 

• “Extra-uterine” (retroperitoneal) endometrial stromal sarcoma 134 

• Heterotopic (retroperitoneal) germ cell tumor 

• “Non-phosphaturic” phosphaturic mesenchymal tumor

272 


B. By clinical behaviour 

• Metastasizing benign fibrous histiocytoma 135 

• Metastasizing benign uterine leiomyoma 136 

• Metastasizing benign meningioma 137 

• (Metastasizing benign mixed tumors of salivary glands) 

C. By macroscopic features 

• “Solid” aneurysmal cyst of soft tissue and bone 

• “Non-protuberans” (atrophic, plaque-type) dermatofibrosarcoma protuberans 

• Encapsulated intradermal dermatofibrosarcoma protuberans 

• “Non-ossifying” ossifying fibromyxoid tumor 

D. By cytological / histological features 

• Cell size / cell type 

i. Desmoplastic small round cell tumor, large cell variant 

ii. Ewing’s sarcoma, large cell (atypical) variant 

iii. Ewing’s sarcoma, pleomorphic/anaplastic variant 137a 

iv. Merkel cell tumor, large cell variant 

v. Anaplastic large cell lymphoma, small cell variant 

vi. Non-pleomorphic spindle-cell atypical fibroxanthoma 

vii. Juvenile xanthogranuloma without multinucleated giant cells 

viii. Plexiform fibrohistiocytic tumor without multinucleated giant cells 

ix. Tenosynovial giant cell tumor without giant cells 

• Cell / matrix peculiarities 

i. Amelanotic melanoma 

ii. Non-lipidized juvenile xanthogranuloma (early phase) 

iii. Sclerotic lipoma 

iv. Fibrous spindle cell lipoma 

v. Extraskeletal myxoid chondrosarcoma without myxoid areas 

vi. Ewing’s sarcoma, schlerosing variant 62a 

• Cell shape 

i. Spindle cell melanoma 

ii. Spindle cell lipoma 

iii. Spindle cell hemangioendothelioma 

iv. Leiomyomatous (spindle cell) rhabdomyosarcoma (both in children and in adults) 

v. 

26a, 67b 

Fibroma-like variant of epithelioid sarcoma 

vi. Ewing’s sarcoma, spindle cell variant 62a 

vii. Spindle cell lymphoma 138 

viii. Sarcomatoid plasmacytoma 

ix. Metastatic spindle cell (fibromatosis-like, sarcomatoid) carcinoma 

• Architectural pattern 

i. Kaposi’s sarcoma (nodular stage) without erythrocyte extravasation 

ii. Solid variant of alveolar rhabdomyosarcoma 

iii. Solid variant of aneurysmal bone cyst 

iv. 

62 62a 

Ewing’s sarcoma with adamantinoma-like pattern 

• Immunohistochemistry 

i. Sarcoma with partial epithelial differentiation (Ewing’s sarcoma 62, 62a, 139 139a-b , fibroblastic dendritic reticulum 

cell sarcoma …) 

ii. Any tumor with divergent/aberrant expression (e.g. keratins in melanoma 140 , lymphoma, vascular neoplasms, 

smooth muscle neoplasms; neuroendocrine markers in sarcoma [chondrosarcoma with neuroendocrine 

differentiation]; …) 

iii. Any tumor with loss of specific antigen (e.g. S-100 protein and HMB-45 negative desmoplastic and nondesmo 

plastic melanoma 85d , cytokeratin negative sarcomatoid carcinoma) 

iv. Non-germ cell tumors expressing germ-cell markers (e.g. PLAP expression in tumors with myogenic 

differentiation and desmoplastic small round cell tumor, ….) 85e


Table XVIII. Cystic tumors and pseudotumors. 

A. Primarily cystic lesions 

• Cutaneous myxoid cyst 

• Myxoid cyst of tendon sheath (ganglion) 

• Nerve sheath ganglion 

• Cavernous lymphangioma (cystic hygroma) 

• Multicystic mesothelioma 

• Lymphangioleiomyomatosis 

B. Lesions with secondary cystic or microcystic change 


• Membranocystic fat necrosis / membranocystic panniculitis / membranous lipodystrophy (primary idiopathic, and 

secondary) 

• Nodular-cystic-encapsulated fat necrosis (“mobile encapsulated lipoma”) 141 

• Lipoma with fat necrosis (including membranous type) 

• Cystic nodular fasciitis 

• Myositis ossificans 


• Perineurioma 

• Myxolipoma 

• Superficial angiomyxoma & juxtarticular myxoma 

• Low grade fibromyxoid sarcoma & hyalinizing spindle cell tumor with giant rosettes 

• Ossifying fibromyxoid tumor (occasional) 

• Giant cell tumor of soft tissue 


• Myxofibrosarcoma 

• Myxoid leiomyosarcoma 


• (Extra-)gastrointestinal stromal tumor 


• Ewing’s sarcoma of the skin (cystic spiradenoma-like tumor) 


C. Pseudocystic pattern 

• Intravenous leiomyomatosis 

• Necrotic sarcomas 

Table XIX. Acral tumors and pseudotumors. 

• Rudimentary supernumerary digit 

• Acquired digital fibrokeratoma 


• Superficial acral fibromyxoma 

• Acral myxoinflammatory fibroblastic sarcoma 

• Cellular digital fibromas 

• Giant cell tumor of tendon sheath – localized-type 

• Glomus tumors 

• Sclerosing perineurioma 

• Hybrid schwannoma-perineurioma tumors 68 


• Chondroma 

• Fibroosseous pseudotumor of the digits 

• Lipofibromatous hamartoma of the nerve (with or without macrodactyly) 141a 

• Nora’s lesion 

• Fibroma of tendon sheath 

• Fibromatosis (palmar: Dupuytren’s disease; plantar: Ledderhose’s disease; penile: Peyronie’s disease) 

• Congenital & infantile fibrosarcoma 


• Epithelioid sarcoma (conventional or “distal” type)

274 


Table XX. Orbital tumors and pseudotumors. 

• Neuromuscular choristoma / hamartoma 


• Periorbital granuloma annulare 


• Intravascular papillary endothelial hyperplasia (Masson’s phenomenon) 

• Amputation neuroma (following enucleation) 

• Amyloid tumor (amyloidoma) 

• Inflammatory pseudotumor 

• Hemangioma 


• Spindle cell lipoma 


• Myolipoma 

• Peripheral nerve sheath tumors 

• Meningioma (lipoblastic type 142 , …) 


• Giant cell angiofibroma 


• Hemangiopericytoma 143 

• Lipomatous hemangiopericytoma (lipomatous solitary fibrous tumor) 144 

• Giant cell tumor (benign osteclastoma) 



• Rhabdomyosarcoma (including sclerosing variant) 


• Mesenchymal chondrosarcoma 

• Granulocytic sarcoma 

• Leukemia / lymphoma 

• Metastatic tumors (sarcoma, chordoma, carcinoma, myoepithelioma, carcinoid, …)


Table XXI. Paratesticular soft tissue tumors and pseudotumors. 

A. Benign tumors and pseudotumors 

• (Adrenal rests) 

• Extrarenal nephrogenic rests 145 

• Angiokeratoma – Fordyce-type, solitary form 

• Angiokeratoma – Fordyce type, multiple, iatrogenic form 146 

• Smooth muscle hyperplasia of testicular adnexa 

• Idiopatic scrotal calcinosis 

• Mechonium periorchitis 

• Sclerosing lipogranuloma 

• Fibromatous periorchitis / nodular periorchitis 

• Granulomatous lymphangitis of the scrotum and penis 

• Granulomatous and nongranulomatous epididymitis 


• Mesothelial hyperplasia 

• Perineal nodular induration in cyclists (3rd testicle / accessory testicle) 

• Fibroma of testicular tunics 

• Proliferative funiculitis 

• Fibromatosis (of spermatic cord) 

• Traumatic neuroma (of the penis) 

• Lipoma 

• Hibernoma 

• Leiomyoma – ordinary (mainly scrotal) & scrotal bizarre leiomyoma 

• Myointimoma (penis) 

• Rhabdomyoma 


• Neurofibroma 

• Fibrous hamartoma of infancy 147 


• Adenomatoid tumor 

• Inflammatory myofibroblastic tumor 

• Calcifying fibrous pseudotumor 

• Angiomyxolipoma 


• Angiomyofibroblastoma-like tumor in males (analogous to vulval cellular angiofibroma) 


• Spleno-gonadal fusion 

• Solitary fibrous tumor (of the spermatic cord) 148 

B. Malignant tumors 

• Melanotic neuroectodermal tumor of infancy 

• Leiomyosarcoma (paratesticular, and penile) 

• Fibrosarcoma 

• Rhabdomyosarcoma (including the spindle cell variant of children) 

• Liposarcoma (mainly well differentiated) 

• Liposarcoma with smooth muscle differentiation) 


• Epithelioid sarcoma (penis) 

• Synovial sarcoma (perineum, penis) 

• Osteosarcoma (penis) 

• Well differentiated papillary mesothelioma of the tunica vaginalis / malignant mesothelioma of the tunica vaginalis 149 

• Paratesticular desmoplastic small round cell tumor

276 


Table XXII. Vulvo-vaginal soft tissue tumors and pseudotumors 150 . 



• Fibroepithelial stromal polyp (bland type; pseudosarcomatous) 50 

151 152 

• Childhood asymmetric labium majus enlargement (aka, prepubertal vulval fibroma) 

• Spindle cell lipoma 


• Cellular angiofibroma 

• Dermatofibrosarcoma protuberans 

• Acquired vulvar lymphangioma (secondary to Crohn’s disease) 

• Vulval angiokeratoma (Fordyce type) 153 


• Angiomyofibrosarcoma (malignant angiomyofibroblastoma) 

• Superficial angiomyxoma 


154 155 

• Post-hysterectomy Fallopian tube prolapse 

• Myoepithelioma – “true” myoepithelioma 

• Adenomyoepithelioma 

• “True” mixed tumor of the vulva 

156 157 

• So-called “mixed tumor” of vagina (spindle cell epithelioma) 

• Lipoblastoma-like tumor 

• Spindle cell / pleomorphic lipoma 

• Atypical lipomatous tumor 158 

• Smooth muscle tumors (any type, including the clear-cell epithelioid leiomyoma of the round ligament 158a ) 

• Genital rhabdomyoma 

• Paraganglioma 159 

• Perivascular epithelioid cell neoplasms 160 




• Melanoma & malignant blue nevus 

• Melanoma with osteocartilaginous differentiation 161


Table XXIII. Aerodigestive tract lesions (involving the orifices). 

A. Nasal cavity 

• Mycobacterial spindle cell pseudotumor 162 


163 164 

• Nasal polyps with atypical stromal cells – a pseudosarcomatous lesion 

• Congenital nasal hemangiopericytoma / infantile myofibromatosis 

• Nasal chondromesenchymal hamartoma in children 39 

• Nasal chondromesenchymal hamartoma in children and adults 130 

• Meningioma 

• Nasal glioma – so-called (nasal cerebral heterotopia or sequestered encephalocele) 


• Olfactory neuroblastoma 

• Sinonasal teratocarcinosarcoma (“mixed olfactory neuroblastoma-craniopharyngioma”) / teratocarcinosarcoma 

(olfactory neuroblastoma with glandular differentiation) 165-167 

• Extramedullary plasmacytoma 

168 169 


170 171 

• Hemangiopericytoma-like tumor (“sinonasal-type hemangiopericytoma”) 

• Nasopharyngeal and extranasopharyngeal 128,128a angiofibroma 

• Smooth muscle tumors 172-174 

• Angiomyolipoma 175 

• Spindle cell myoepithelioma 176 

• Undifferentiated small cell tumor 177 

• Alveolar rhabdomyosarcoma (paranasal sinuses) 



B. Oral cavity 

• Rhabdomyomatous mesenchymal hamartoma 178 

• Fibrous hamartoma of infancy 179 

• Congenital granular cell epulis 


• Neuroma 



• Rhabdomyoma 

• Multifocal rhabdomyoma 180 

• Ectomesenchymal chondromyxoid tumor of the tongue 


• Fibrolipoma 

• Ordinary lipoma 

• Spindle cell lipoma 181 

• Pleomorphic hyalinizing angiectactic tumor 182 

• Peripheral myxoma 183 

184 185 


• Congenital hemangiopericytoma / infantile myofibromatosis 

• Epithelioid hemangioendothelioma 186 





• Clear cell sarcoma 187 

• Lingual alveolar soft part sarcoma 188 

• Low-grade malignant Triton tumor 189 

• Synovial sarcoma 180 

• Ordinary melanoma 

• Melanoma with osteocartilaginous differentiation 161 

C. Anal canal* 

• CD34+ fibroepithelial polyp 191 


• Schwannoma & neurofibroma 

• Leiomyoma 192 

• Leiomyosarcoma 192 

192a, 192b 

• Gastrointestinal stromal tumor

278 


Table XXIV. Intranodal primary tumors & pseudotumors (other than hematolymphoid). 

• Nodular fasciitis (of the capsule) 


• Deciduosis 

• Lipomatosis 


• Lymphangioleiomyomatosis 

• Inflammatory pseudotumor 

• Mycobacterial spindle cell pseudotumor 


• Rosai-Dorfman disease 

• Castleman’s disease (hyaline-vascular type)-related tumors (follicular dendritic cell tumor, Kaposi’s sarcoma) 

and pseudotumors (angiolipomatous hamartoma) 

• Angiomyomatous hamartoma 


• Angiolymphatic angiodysplasia 

• Vascular transformation of sinuses 

• Nodular spindle-cell vascular transformation of lymph nodes 193 

• Nodal angioma (common type – capillary, cavernous, lobular, cellular) 



• Spindle and epithelioid hemangioendothelioma 

• Polymorphous hemangioendothelioma 

• Angiosarcoma 


• Leiomyoma 

• Palisaded myofibroblastoma 


• Myelolipoma


Table XXV. Mammary stroma spindle cell tumors and tumor-like lesions. 

A. Monomorphic spindle cell tumors and tumor-like lesions of specialized mammary stroma 194-196 

• Pseudoangiomatous stromal hyperplasia 

• Benign spindle cell tumor, NOS 

• Spindle cell lipoma-like tumor 


• Myofibroblastoma 

• Combined spindle cell tumor (two of the above: e.g., myofibroblastoma plus solitary fibrous tumor areas 

or spindle cell lipoma-like areas) 

• Periductal stromal tumor 

• Stromal sarcoma 

B. Monomorphic spindle cell tumors and tumor-like lesions of non-specialized mammary stroma 


• Lipoma 

• Muscular hamartoma 



• Leiomyoma 

• Inflammatory myofibroblastic tumor (benign, low-grade) 

• Spindle cell adult type juvenile xanthogranuloma 197 


• Fibrosarcoma 

• Myofibrosarcoma 


• Peripheral nerve sheath tumor (benign and malignant) 

• Monophasic fibrous synovial sarcoma 


• Mammary NOS-type sarcoma with CD10 expression and features of myoepithelial differentiation 198 

C. Spindle cell myoepithelioma 


D. Spindle cell epithelial tumors 

• Metaplastic carcinoma – biphasic and monophasic 199 

• Monophasic low-grade spindle cell fibromatosis-like carcinoma 122, 199 ) 

• Monophasic high-grade spindle cell (sarcomatoid) carcinoma (including a subset showing myoepithelial 

differentiation) 199a

280 


Table XXVI. Infantile-juvenile tumors and pseudotumors. 

A. Hamartomas – Developmental lesions 

• Connective tissue nevi 

• Palmar cutaneous hamartoma 

• Nevus lipomatosus superficialis 

• Cutaneous neural heterotopia 

• Cutaneous ganglion cell choristoma 

• Hamartomatous neuroma combined with heterotopic ganglion cells 

• Congenital neural hamartoma (“fascicular schwannoma”) 

• Multiple cutaneous neuromuscular choristomas 

• Pleomorphic neuromuscular hamartoma of the subcutis (superficial equivalent of deep neuromuscular hamartoma 

or benign Triton tumor) 

• Congenital neurovascular hamartoma of the skin 


• Congenital smooth muscle hamartoma of the skin 

• Genital smooth muscle hamartoma 

• Rhabdomyomatous mesenchymal hamartoma 

• Striated muscle hamartoma 

• Neuromuscular hamartoma / choristoma (benign Triton tumor) 

• Neural fibrolipomatous hamartoma 

• Ectopic meningothelial hamartoma of the scalp 

• Sequestrated (rudimentary) meningocele 

• Sacro-coccygeal (“myxopapillary”) ependymal rests 

• Meningo-glial nodule of the buttock 

• Soft tissue gliomatosis in a non-midline location 

• Neurocristic cutaneous hamartoma 

39 130 

• Nasal chondromesenchymal hamartoma 

• Mesenchymal hamartoma of the chest wall 40 

• Lipomatous and angiomatous lesion associated with occult spinal dysraphism 

B. Benign tumors and pseudotumors 

• Granular (gingival) cell tumor 



• Infantile fibromatosis, diffuse and desmoid type 




• Juvenile hyaline fibromatosis 22 / infantile systemic hyalinosis / and Winchester syndrome 



• Cranial fasciitis 

• Fibrous umbilical polyp (fasciitis-like proliferation of infancy) 200 

• Umbilical pseudotumors (keloid/hypertrophic scar, umbilical polyp/granuloma with urachal, omphalomesenteric, or 

other remnants) 

• Lipoblastoma / lipoblastomatosis 

• Lipofibromatosis 

• Myositis ossificans progressiva (fibrodysplasia) 

• Benign and atypical fibrous histiocytoma 


• Juvenile xanthogranuloma 

• Fetal rhabdomyoma 

• Fetal rhabdomyoma, cellular-type 

• Juvenile rhabdomyoma 

• Cellular capillary hemangioma 

• Intramuscular hemangioma 

• Kaposi-like infantile hemangioendothelioma 

• Spindle cell hemangioendothelioma 

• Congenital or infantile hemangiopericytoma 

• Congenital giant cell angioblastoma 

• Nasopharyngeal and extra-nasopharyngeal angiofibroma 171a, b 

• Congenital and childhood plexiform (multinodular) cellular schwannoma 110


C. Intermediate and malignant tumors 

• Congenital myofibromatosis (visceral involvement) 

• Congenital (infantile) fibrosarcoma 

• Kaposi’s sarcoma (HIV-negative, peripartum HHV-8 infection) 

• Lymphangioendotheliomatosis 

• Primitive myxoid mesenchymal tumor 200a 

• Pigmented (melanotic) neuroectodermal tumor of infancy 

(maxilla, mandible, mediastinum, skull, epididymis, soft tissue of extremities) 

• Inflammatory pseudotumor – Inflammatory myofibroblastic tumor 


• Pediatric dermatofibrosarcoma protuberans (rare; occasionally congenital) 

• Pediatric pigmented dermatofibrosarcoma protuberans 200b 

• Pediatric leiomyosarcoma 

• Endovascular papillary angioendothelioma (Dabska’s tumor) 

• Ewing’s sarcoma 

• Congenital Ewing’s sarcoma 

• Primitive neuroectodermal tumor 

• Rhabdomyosarcoma (embryonal, alveolar; rarely congenital) 

• Infantile rhabdomyofibrosarcoma 

• Synovial sarcoma (even congenital) 

• Congenital angiomatoid malignant fibrous histiocytoma 

• Congenital synovial sarcoma 

• Congenital extraskeletal embryonal chondrosarcoma 



• Congenital disseminated extrarenal malignant rhabdoid tumor 

• Angiomatoid (malignant) fibrous histiocytoma 

• Pediatric leiomyosarcoma of soft tissue (rare) 

• Pediatric epithelioid sarcoma (rare) 201 

• Pediatric malignant peripheral nerve sheath tumor (rare) 202 

• Pediatric malignant vascular tumors 203 

• Liposarcoma (exceptional) 

• Pediatric clear cell sarcoma (of tendons and aponeurosis) 204 

• Primitive undifferentiated sarcoma 

• Congenital leukemia cutis 

• Cutaneous neonatal neuroblastoma 

• Soft tissue sarcoma as a second malignant neoplasm 205

282 


Table XXVII. Lesions as sentinel of clinical entities (see also Table XXVIII). 

• Keloid (acne vulgaris; ear-piercing; previous surgical intervention or thermal injuries) 

• Membranocystic fat necrosis / membranocystic panniculitis / membranous lipodystrophy (secondary form - 

mainly lower leg venous insufficiency in obese women, erythema nodosum, morphea profunda, 

lupus panniculitis, dermatomyositis, subcutaneous T-cell lymphoma, factitial ulcer, … others) 

• Nodular panniculitis (Weber-Christian disease; alpha-1-antitrypsin-deficiency; Rothmann-Makai panniculitis; 

pancreatic disease) 

• Massive localized lymphedema (morbid obesity) 

• Polyvinylpyrrolidone granuloma (polymer used as plasma expander in a previous surgical intervention or as 

vehicle for intravenous medications) 

• Pseudosclerodermatous panniculitis of the breast (previous radiation therapy for breast cancer) 206 

• Pseudolipoma (“battered buttock syndrome”) 

• Folded skin with lipomatous nevus (“Michelin-tire baby”) 

• Multiple nevus lipomatous cutaneous superficialis (Hoffman-Zurhelle disease) 

• Granular histiocytic granuloma (local previous site of surgery) 

• Scleredema (maturity-onset diabetes mellitus) 

• Xanthogranulomatous pseudotumor (Erdheim-Chester disease) 

• Superficial fibromatoses (association of palmar with plantar and/or penile forms) 

• Multiple desmoids tumors (familial infiltrative fibromatosis) 

• Myofibroma (myofibromatosis – bone, and visceral organ involvement; possible familial occurrence) 

• Kimura’s disease (minimal change/membranous glomerulopathy nephrotic syndrome; peripheral blood 

eosinophilia; elevated serum IgE) 

• Paraffinoma – oleogranuloma (cosmetic purposes [breast of females, breast of transsexual males, eyelids, lips, male 

genitalia – scrotum, penis]; pseudotherapeutic and therapeutic procedures (scalp [for baldness], sinonasal surgery 

and medication, intralesional injection of oil based chemotherapeutic agents) 

• Gingival pyogenic granuloma (granuloma gravidarum) (pregnancy) 

• Soft tissue and cutaneous deciduosis (pregnancy; occasionally in non-pregnant women) 207 

• Leiomyomatosis peritonealis disseminata (excess exogenous or endogenous sex steroids, pregnancy, endometriosis) 

• Extramedullary hematopoiesis (anemia of various origin, chronic myeloproliferative disorders) 

• Extramedullary myeloid tumor (acute or chronic myelogenous leukemia) 

• Sclerosing extramedullary hematopoietic tumor (chronic myeloproliferative disorders) 

• Extramedullary plasmacytoma (full-blown multiple myeloma) 

• Cytophagic histiocytic panniculitis (subcutaneous T-cell lymphoma) 

• Angiosarcoma (preexisting lymphedema, previous local irradiation 208 , local lymphedema, …) 

• Atypical vascular lesion of skin of the breast (previous irradiation) 

• Mucosal, lymph nodal and/or visceral Kaposi’s sarcoma (immunosuppression, AIDS) 

• Verruga peruana / bacillary angiomatosis (Carrion disease / AIDS) 

• Acquired progressive lymphangioma (irradiation, trauma, …) 

• Epithelioid hemangioma (multicentricity) 

• Multicentric reticulohistiocytosis (paraneoplastic disease) 

• Xanthoma – various types (e.g. tendinous xanthoma: disorders of lipid metabolism) 

• Plexiform xanthomatous tumor (hyperlipidemia) 

• Calcinosis circumscripta and calcinosis universalis (scleroderma, dermatomyositis, Raynaud disease, 

hyperparathyroidism,…) 

• Ectopic calcifications (hypercalcemia-associated conditions: milk-alkali-syndrome or Burnett syndrome, 

hypervitaminosis D, tumoral bone metastases) and calciphylaxis 209 (associated with secondary hyperparathyroidism 

/ chronic renal failure) 

• (Idiopathic) tumoral calcinosis (unassociated with hypercalcemia: sometimes hyperphosphatemia-associated; 

sometimes familial) 

• (Secondary) tumoral calcinosis-like lesion (associated with chronic renal failure and secondary hyperparathyroidism) 210 

• Soft tissue calcifications and osteomas (Albright hereditary osteodystrophy) 

• Cutaneous and subcutaneous myxoma (Carney's complex) 

• Pretibial myxedema (hyperthyroidism) 

• Dermatofibrosarcoma protuberans (familial form) 

• Multiple lipomas (familial form) 

• Multiple spindle cell lipomas (familial form) 

• Multiple angiolipomas (familial form) 

• Symmetric lipomatosis of the head and neck (Madelung disease or Launois-Bensaude syndrome) 

• Asymmetrical lipomatosis with “buffalo hump” (steroid lipomatosis - Cushing's disease) 

• Asymmetrical lipomatosis with wasting face and limbs (iatrogenic HIV lipodystrophy)


• Pelvic lipomatosis (mostly affecting blacks) 

• Adiposis dolorosa (Dercum's disease) 

• Multiple scwhannoma (schwannomatosis) 109b-d 

• Multiple cutaneous chondromas (familial form) 

211 212 

• Amyloid tumor (plasma cell dyscrasia; chronic inflammatory/autoimmune diseases; chronic hemodyalisis; others) 

• Pseudoneoplastic parathyromatosis following iatrogenic implantation (hyperparathyroidism) 

• Phosphaturic mesenchymal tumor (oncogenic osteomalacia/rickets) 

• Idiopathic retroperitoneal fibrosis – Ormond’s disease (association with other fibrosclerotic disorders, e.g. Riedel 

thyroiditis, primary sclerosing cholangitis, sclerosing mediastinitis, inflammatory pseudotumor of the orbit, 

autoimmune pancreatitis) 

47 48 

• Tamm-Horsfall protein deposition / “retroperitoneal urinary precipitates” (past urine extravasation) 

• Hemangiopericytoma & solitary fibrous tumor (hypoglycemia) 213 

• (Retroperitoneal) extrarenal juxtaglomerular cell tumor (secondary hyperaldosteronism – hypertension, 

hypokalemia) 

• Angiomyolipoma & lymphangioleioleiomyomatosis (tuberous sclerosis complex) 

• Malignant Triton tumor and epithelioid malignant peripheral nerve sheath tumor (neurofibromatosis I) 

• Paragangliomas (both head and neck and retroperitoneal) – either alone (PGL1, PGL2, PGL3, PGL4 syndromes) or as 

a component of a multiple tumor syndrome (von Hippel-Lindau disease, neurofibromatosis type 1, MEN-2b, 

Carney's triad, Carney' and Stratakis' syndrome 214 or in association with gastrointestinal stromal tumor 214a ) 

• Gastrointestinal stromal tumor (familial form, and syndromic form in association with lentigines and mast cell 

tumors) 192b 

• Multifocal (EBV-associated) smooth muscle tumors (post-transplantation 215 ; congenital immunodeficiencies) 

• Multifocal visceral smooth muscle tumors (immunocompromised HIV-patients) 

• Myxoid embolus (cardiac myxoma) 

• Sarcomatous embolus (great vessel sarcoma) 216

284 


Table XXVIII. Lesions as sentinel of clinical syndromes (see also Table XXVII). 

• Angiofibroma of the face (tuberous sclerosis complex) 

• Periungual, subungual, and gingival angiofibroma (tuberous sclerosis complex; Gardner syndrome) 

• Fibrofolliculoma (Birt-Hogg-Dube syndrome) 217 

• Fibrous papule of the face (multiple hamartoma syndrome; Cowden’s syndrome) 

• Cutaneous myxoma (mainly of external ear location) (LAMB / NAME / Carney’s complex) 

• Gardner-associated fibroma (Gardner syndrome; familial infiltrative fibromatosis; desmoid fibromatosis) 

• Nuchal-type fibroma (diabetes mellitus; Gardner syndrome) 

• Desmoid tumor (familial adenomatous polyposis / Gardner syndrome) 

• Nasopharyngeal angiofibroma (familial adenomatous polyposis / Gardner syndrome) 

• Keloids (Rubinstein-Taybi syndrome; Ehlers-Danlos syndrome) 

• Gingival fibromatosis (Zimmermen-Laband syndrome; Ramon syndrome; Klippel-Trenaunay syndrome; Cowden’s 

syndrome; prune-belly syndrome) 

• Juvenile hyaline fibromatosis and infantile systemic hyalinosis (Winchester syndrome) 

• Gingival fibromatosis with juvenile hyaline fibromatosis (Murray-Puretic-Drescher syndrome) 

• Penile fibromatosis (Cogan syndrome) 

• Cerebriform superficial soft tissue fibrous proliferation (Proteus syndrome) 

• Multiple lipomas (Proteus syndrome) 

• Multiple intramuscular myxoma plus polyostotic fibrous dysplasia (Mazabraud’s syndrome) 

• Lymphangioma (Turner syndrome) 

• Lipomas and hemangiomas (Bannayan-Ruvalcaba-Riley syndrome; Cowden syndrome) 

• Multiple lipoma (Bannayan-Zonana syndrome; Frohlich syndrome; Proteus syndrome) 

• Kaposiform hemangioendothelioma (Kasabach-Merritt syndrome) 

• (Large) cavernous hemangioma (Kasabach-Merritt syndrome) 

• Cutaneous and gastrointestinal venous malformations (blue rubber bleb nevus syndrome) 

• Embryonal rhabdomyosarcomas (Beckwith-Wiedemann syndrome / Exomphalos-macroglossa-gigantism 

syndrome) 

• Soft tissue sarcomas (retinoblastoma syndrome) 

• Soft tissue sarcomas (Werner syndrome) 

• Myogenic sarcomas (Rubinstein-Taybi syndrome) 

• Pedal hemangioma (Turner syndrome) 

• Hemangiomatosis (Klippel-Trenaunay syndrome) 

• Angiomatous lesions (Sturge-Weber syndrome; Klippel-Trenaunay syndrome; Parkes-Weber syndrome; 

Rendu-Osler-Weber disease) 

• Multiple hemangiomas and chondromas (Maffucci’s syndrome) 

• Spindle cell hemangioma (Maffucci’s syndrome – occasional) 

• Angiosarcoma, chondrosarcoma (Maffucci’s syndrome) 

• Multiple glomus tumors and/or multiple glomus body hamartomas (congenital, familial) 

• Glomeruloid hemangioma (POEMS / Crowe-Fukase / PEP / Takatsuki syndrome) 

• Hemangioblastoma of soft tissue (von Hippel-Lindau syndrome) 

• Lymphangioleomyomatosis / soft tissue PEC-oma (tuberous sclerosis complex) 

• Major and/or minor (2 or more) features of tuberous sclerosis complex plus autosomal dominant polycystic 

kidney disease (adjacent gene syndrome TSC2/ADPKD1 217a ) 

• Lymphangiosarcoma (Stewart-Treves syndrome) 218 

• Mucosal neuromas (hypertrophied corneal nerves, bumpy lips, nodular tongue in MEN-2b) 

• Plexiform neurofibroma and multiple neurofibromas (neurofibromatosis type I) 218a 

• Bilateral acoustic schwannoma (neurofibromatosis type II) 

• Calcinosis circumscripta and calcinosis universalis (CREST syndrome; or Thibierge-Weissenbach syndrome [calcinosis, 

Raynaud's phenomenon, eophageal dysfunction, sclerodactyly, and telangiectasia]; milk-alkali syndrome [Burnett 

syndrome]; abnormalities of calcium and phosphate metabolism) 

• Psammomatous melanotic schwannoma (Carney’s complex) 

219 220 

• Fetal rhabdomyoma (nevoid basal-cell carcinoma syndrome – Gorlin-Goltz syndrome) 

219 220 

• Ectopic calcifications (nevoid basal-cell carcinoma syndrome – Gorlin-Goltz syndrome) 

• Intraabdominal (mesenteric) and extrabdominal fibromatosis (Gardner syndrome) 

• Idiopathic retroperitoneal fibrosis – Ormond’s syndrome (immune-mediated connective tissue disease complex: 

see above at Table 27) 

• Malignant peripheral nerve sheath tumors (neurofibromatosis type 1) 

• Rhabdomyosarcoma (Costello syndrome) 

• Rhabdomyosarcoma, perianal (Nijmegen breakage syndrome) 

• Malignant rhabdoid tumor (rhabdoid tumor predisposition syndrome) 221 

• Multiple sarcomas (e.g. rhabdomyosarcoma) (Li-Fraumeni syndrome and Li-Fraumeni-like syndrome) 222


Table XXIX. Embryological rests or normal anatomic structures potentially mistaken 

for tumors. 

A. Normal embryological rests in soft tissue 

• Branchial arch-derivatives (may mimic soft tissue chondroma) 

• Branchial pouch-derivatives (may mimic metastatic carcinoma, …) 

• Supernumerary breast 

• Lumbosacral ectopic nephrogenic rest 223 224 (may mimic metastatic or ectopic Wilms’ tumor, monodermal 

teratoma, …) associated and unassociated with spinal dysraphism 

• Sacrococcygeal ependymal rests (resembles ependymal tumors, metastatic papillary carcinoma, …) 

• Odontogenic epithelial rests 

B. Normal anatomic structures in appropriate locations 

• (Dental papilla [resembling myxoma]) 

• Accessory tragus-elastic cartilage (resembling chondroma) 

• Glomus coccygeum (resembling glomus tumor) 

225 226 

• Organ of Zuckerkandl hypertrophy (resembling paraganglioma) 

227 228 

• Carotid body hyperplasia (resembling paraganglioma) 

• Chiewitz organ (resembling metastatic carcinoma) 229-231 

• Pacinian corpuscle hyperplasia (resembling Pacinian neuroma / Pacinian neurofibroma - Pacinian perineurioma / 

onion-bulb neuropathies) 232-233 

C. Normal anatomic structures in inappropriate locations 

• Dermal thymus (brachio-oculo-facial syndrome) (may mimic thymoma) 234 

• Glial heterotopia (may mimic glioma) 

• Hamartoma of the scalp with ectopic meningothelial elements / rudimentary meningocele (may mimic 

various neoplasms, e.g. angiosarcoma, meningioma, …) 

• Extramedullary hemopoiesis in normal organs (soft tissue, breast, lymph node, brain, …) (may mimic lympho- or 

myelo-proliferative processes) 

• Extramedullary hemopoiesis in vascular tumors (hemangioblastoma, spindle cell lipoma, pyogenic granuloma,...) 

(may mimic lymphoid or myeloid leukemia) 

• Subcutaneous splenosis (resembles hematologic proliferations) 

• Langerhans’ cell islets in adipose peripancreatic tissue (resembles metastatic carcinoid) 

• Discontinuous splenogonadal fusion 

• Any other adult tissue ectopically located in soft tissue (e.g. post-traumatic splenosis in the abdomen, 

autografted parathyroid in the forearm 11/ix-5 , …)

286 


Table XXX. Categorization i-iv of soft tissue tumors and its relevance to management. 

i 

ii 

iii 

iv 

Pathologic categorization is defined on the basis of the clinical behaviour of each entity. 

This clinically-based categorization of soft tissue tumors broadly corresponds to that found in specialized standard 

textbooks 235-237 and major or specific publications. 

The range of metastatic rates given within each category/group is an approximation gauged from the literature and from 

the authors’ experience, and – although it reflects the basis for the categorization – should be interpreted as approximate 

guide only. 

Sarcomas are stratified according to a 3-tiered prognostic scheme, intended as a “working formulation” and not as a grading 

system along the lines of existing traditional grading schemes such as FNCLCC (see below). This stratification conveys notable 

differences in metastatic rate. 

• Category I. Clinically benign tumors 

Metastatic rate of nearly 0%. Local excision is usually curative 

Dermatofibroma (including variants – clear cell, epithelioid, sclerosing, myxoid, keloidal, palisading, lipidized, …) & cutaneous benign 

fibrous histiocytoma^, fibroma (including variants – sclerotic, pleomorphic, nuchal-type, Gardner-associated type, …), fibroma 

of tendon sheath, giant cell tumor of tendon sheath (localized-type), superficial fibromatosis (prone to recurrence) °, deep 

fibromatosis/desmoids tumor (prone to recurrence) °, nasopharyngeal angiofibroma (prone to recurrence)°, desmoplastic fibroblastoma 

(collagenous fibroma), mammary-type fibroblastoma, giant cell fibroblastoma (may recur), intranodal palisaded 

myofibroblastoma, dermatomyofibroma, myofibroma adult-type, myofibromatosis infantile-type (“infantile hemangiopericytoma”), 

intramuscular myxoma (including the cellular variant), calcifying fibrous pseudotumor, calcifying aponeurotic fibroma 

(prone to recurrence), juxtaarticular myxoma, cutaneous myxoma, superficial angiomyxoma with and without epithelial elements 

(prone to recurrence) 238 , “aggressive” angiomyxoma of the pelvis (prone to recurrence) + , vulvovaginal and inguinoscrotal 

angiomyofibroblastoma § , nasopharyngeal angiofibroma (prone to recurrence; locally aggressive), vulval cellular angiofibroma 

(and its analogous counterpart “angiomyofibroblastoma-like tumor” in males), localized tenosynovial giant cell tumor, 

cutaneous leiomyomas (pilar-type, vascular-type, genital-type) and variants (atypical/symplastic, pleomorphic, clear cell, granular 

cells, …), leiomyoma of deep soft tissue 242 243 , myointimoma (little experience), rhabdomyoma (including variants – fetal type, oncocytic, 

genital, …), “usual” glomus tumor (including some variants – glomangioma, glomangiomyoma, glomangiomatosis; intravascular; 

oncocytic, …, but excluding “unusual” glomus tumors: see at borderline and malignant groups), myopericytoma∞, 

glomangiopericytoma, lipoma (including variants: multiple, deep-seated, pleomorphic, spindle cell, fibrohistiocytic, chondroid, 

…), lipoblastoma, lipofibromatosis (may recur), hibernoma, myolipoma, angiolipoma (including the cellular variant), fibrolipoma, 

dendritic fibromyxolipoma, angiomyxolipoma, endothelial neoplasms (capillary hemangioma and variants, cavernous hemangioma, 

hobnail hemangioma, acquired elastotic hemangioma, epithelioid hemangioma, glomeruloid hemangioma, spindle cell 

hemangioma, Kaposiform hemangioendothelioma; angiomatosis; lymphangioma; acquired progressive lymphangioma – benign 

lymphangioendothelioma, lymphangiomatosis) 245 246 , soft tissue hemangioblastoma (little experience so far), ordinary schwannoma 

(including variants – epithelioid, pseudoglandular, pigmented, neuroblastoma-like, ancient, lipomatous, sclerosing, …, but 

excluding the psammomatous melanotic type), cellular schwannoma (may recur, does not metastasize) ° 115 247 , neurofibroma 

(including typical variants – diffuse, plexiform, dendritic cell, pigmented, sclerosing, lipomatous, …; cellular and/or atypical variants 

may recur) °, soft tissue perineurioma (7/V-5, 248) (including typical variants – intraneurial, myxoid, reticular, Pacinian, sclerosing, 

…- and atypical forms ⊃ ), hybrid or composite nerve sheath tumors (neurofibroma/schwannoma, neurofibroma/perineurioma, 

schwannoma/perineurioma), usual granular cell tumor (Abrikossof tumor), benign Triton tumor, ganglioneuroma° and ganglion 

cell tumor, neurothekeoma (myxoid-type, cellular-type), soft tissue chondroma, fibrochondroma, myxochondroma, osteoma, 

and osteochondroma (including variants – multiple, familial), extraarticular synovial chondromatosis, soft tissue oncocytoma 

(little experience), hyaline cell-rich chondroid syringoma, ectopic hamartomatous thymoma √ , myelolipoma. 

^ Some variants – atypical, cellular, aneurysmal, deepseated – may recur and anecdotal metastatic cases 135 are also on record. ° May undergo malignant 

change. + 2 metastasizing cases on record 239 240 . § 1 case of malignant transformation 241 . ∞ Malignant myopericytomas are also on record 244 . 

⊃ 

Malignant perineuriomas likely correspond to malignant peripheral nerve sheath tumors with perineurial differentiation 249-251 . √ 1 case of malignant 

transformation 252 . 

Note: tumors “prone to recurrence” might be also placed in Group II (see footnote there).


• Category II. Clinically intermediate !!! (borderline) tumors 

Metastasis can rarely occur: metastatic rate of < 5%. 

Prone to recurrence if incompletely excised: attention must be paid to margins. 

Adjuvant therapy not warranted. 

Tumor may transform or dedifferentiate to group III (below). 

Atypical fibroxanthoma (any variant – spindle cell nonpleomorphic, pigmented or aneurysmal, clear cell, granular cell, osteoclastic 

giant cell, …), “some” cutaneous and subcutaneous “benign” fibrous histiocytomas (histology-dependent – atypical, cellular, 

aneurysmal; site-dependent – deep-seated, head & neck area; previous excision – recurring lesions) 135 253 , dermatofibrosarcoma 

protuberans° (classic-type, Bednar/pigmented type – any site, any age, any variant), hybrid dermatofibrosarcoma protuberans/giant 

cell fibroblastoma°, plexiform fibrohistiocytic tumor, giant cell angiofibroma, solitary fibrous tumor° (including 

lipomatous hemangiopericytoma), atypical lipomatous tumor°, angiomatoid fibrous histiocytoma (aka, angiomatoid malignant 

fibrous histiocytoma), ossifying fibromyxoid tumor°, inflammatory myofibroblastic tumor (“inflammatory pseudotumor”)°, 

“inflammatory fibrosarcoma”, pleomorphic hyalinizing angiectatic tumor – hemosiderotic fibrohistiocytic lipomatous tumor 

(prone to recurrence) 254 , myxoinflammatory fibroblastic sarcoma, deep-seated or large sized benign appearing smooth 

muscle tumors of soft tissue 243 243a , well-differentiated liposarcoma (lipoma-like variant, sclerosing variants), “atypical” glomus tumors 

(deep-seated only / superficially located & mitotically active only / large-sized only) 255 , endothelial neoplasms (Dabska’s tumor 

or papillary intralymphatic angioendothelioma, retiform hemangioendothelioma, composite hemangioendothelioma, 

polymorphous hemangioendothelioma, giant cell angioblastoma, Kaposi’s sarcoma) 245 246 , giant cell tumor° (skin, soft tissue, 

breast) 256 , phosphaturic mesenchymal tumor° (excluding atypical variants), superficial low-grade fibromyxoid sarcoma (may 

recur) 257 , diffuse-type tenosynovial giant cell tumor (“pigmented villonodular synovitis/bursitis”) (prone to recurrence)°, soft tissue 

mixed tumor, myoepithelioma, parachordoma. 

!!! 

“Intermediate” is a term which is also used for tumors which can recur but do not metastasize; these are also (more appropriately) termed locally 

aggressive. ° May undergo sarcomatous change. 

• Category III (X, Y, Z groups). Clinically malignant tumors 

Local excision with critical attention to margins (X, Y, Z). 

Consideration given to adjuvant therapy (Y). 

Adjuvant therapy usual (Z). 

Group III-X. Low-grade malignancy – metastatic rate of 5-10%. 

Infantile/congenital fibrosarcoma, well-differentiated (fibromatosis-like / Broders gr. I) fibrosarcoma, low-grade fibromyxoid sarcoma 

§ (including its variant hyalinising spindle cell tumor with giant rosettes), gr. I malignant fibrous histiocytoma (“low-grade” π 

myxofibrosarcoma), angiomatoid (malignant) fibrous histiocytoma, cutaneous leiomyosarcoma, usual / well differentiated solitary 

fibrous tumor, low-grade myofibrosarcoma, well-differentiated leiomyosarcoma, leiomyosarcoma in children, well differentiated 

liposarcoma with leiomyosarcomatous differentiation (w.d. lipoleiomyosarcoma), spindle cell liposarcoma, “lowgrade” 

dedifferentiated liposarcoma 72 , psammomatous melanotic schwannoma, low-grade malignant peripheral nerve 

sheath tumor, well-differentiated (gr. I) chondrosarcoma, low-grade angiosarcoma 245 246 . 

§ 

Ungradable according to some. 

π 

Note: here are two tiered grading systems: one is 2-tiered (low- and high-grade) and the other is 3-tiered (low-, intermediate-, and high-grade). 

Group III-Y. Intermediate-grade malignancy – metastatic rate 10-30% 

Fibrosarcoma arising in dermatofibrosarcoma protuberans (classic-type, Bednar-type) ψ , moderately differentiated conventional 

fibrosarcoma, solitary fibrous tumor (atypical, malignant), “true” well-differentiated malignant hemangiopericytoma 261 , gr. 

II myxoid malignant fibrous histiocytoma (“high-grade” π myxofibrosarcoma), storiform malignant fibrous histiocytoma, conventional 

myxoid liposarcoma, conventional leiomyosarcoma, myxoid leiomyosarcoma, granular cell leiomyosarcoma (little experience), 

spindle cell embryonal rhabdomyosarcoma (“leiomyomatous variant”), moderately differentiated malignant peripheral nerve sheath 

tumor, conventionally dedifferentiated liposarcoma, malignant mesenchymoma, epithelioid hemangioendothelioma ∆ , “lowgrade” 

extraskeletal myxoid chondrosarcoma 262 and its analogous variant with neuroendocrine differentiation, soft tissue 

follicular dendritic cell sarcoma. 

ψ 

Metastatic rate of 10-30% 258-260 . π We have used a 2-tiered grading system for myxoid malignant fibrous histiocytoma (Angerwall’s myxofibrosarcoma). 

∆ Classic histologies correlate with the lower end of metastatic rate of the category, while atypical features correlate with the upper end of 

the metastatic prognostic spectrum.

288 


Group III-Z. High-grade malignancy – metastatic rate > 30%. 

Poorly differentiated conventional fibrosarcoma, sclerosing epithelioid fibrosarcoma, high grade myxoid/round cell liposarcoma, 

epithelioid liposarcoma, pleomorphic liposarcoma ≠ , some mixed-type liposarcoma (myxoid/round cell and pleomorphic liposarcoma), 

pleomorphic myofibrosarcoma, “true” conventional malignant hemangiopericytoma, poorly differentiated leiomyosarcoma, 

pleomorphic leiomyosarcoma, leiomyosarcoma, pleomorphic leiomyosarcoma, leiomyosarcoma with prominent osteoclastic giant 

cells, epithelioid leiomyosarcoma ≠ , inflammatory leiomyosarcoma, rhabdomyosarcoma ↑↑↑ ≠ (any type – embryonal, alveolar, 

pleomorphic), angiosarcoma ↑↑↑ ≠ (any type – conventional, epithelioid, poorly differentiated) 245 246 , intimal sarcoma, glomangiosarcoma 

≠ 255 263 , malignant peripheral nerve sheath tumor (poorly differentiated, epithelioid, pleomorphic) ≠ , malignant granular cell tumor 

264 

, malignant Triton tumor, conventional epithelioid sarcoma (“distal-type”, classic form ≠ § and variants 26a,67a,67b – fibroma-like, angiectoid), 

proximal-type epithelioid sarcoma, extra-renal malignant rhabdoid tumor ↑↑↑ ≠ , synovial sarcoma ≠ § , mesenchymal 

chondrosarcoma ↑↑↑ , “high-grade” extraskeletal myxoid chondrosarcoma 262 and its analogous variant with neuroendocrine differentiation, 

extraskeletal osteosarcoma ↑↑↑ ≠ (any type – conventional and giant-cell rich), alveolar soft part sarcoma § , clear cell sarcoma 

§ , soft tissue PEComas (monotypic epithelioid angiomyolipomas) ≠ , Ewing’s sarcoma / primitive neuroectodermal tumor ↑↑↑ ≠ , 

desmoplastic small round cell tumor ↑↑↑ , high grade malignant fibrous histiocytoma group (pleomorphic malignant fibrous histiocytoma 

[undifferentiated pleomorphic sarcoma], osteoclastic giant cell malignant fibrous histiocytoma [undifferentiated pleomorphic 

sarcoma with osteoclastic giant cells], inflammatory malignant fibrous histiocytoma / undifferentiated pleomorphic sarcoma with 

prominent inflammation). 

≠ 

Primary analogue high grade tumors also described in cutaneous site 2 , for which the predicted prognosis should not be applied. ↑↑↑ Definitionally 

high grade sarcomas. § May give rise to metastasis after a long duration follow-up, despite the absence of local recurrence. 

Clinical and Pathological Parameters influencing Prognosis (“Prognostic Predictors”) 265-272 

235-237 243 243a 271 

Clinicopathologic features – e.g. anatomical sites (abdominal cavity, retroperitoneum, head-neck worse than 

limbs, and abdominal and chest wall); tumor location (deep soft tissue worse than skin and subcutis); tumor size (the larger the 

worse); patient’s age (old age generally more adverse than young); clinical setting (secondary sarcomas arising in benign tumors, 

immunosuppression-associated tumors, virus-induced tumors, …); others … 

Grading systems and histological grades 265-271 – e.g. grade I, II, III according to the French/FNCLCC grading system 266-268 , which 

evaluates 3 independent parameters (tumor differentiation, mitosis count, and necrosis) each scored 1 to 3: grade I → 2-3; grade 

II → 4-5; grade III → 6-8. 

Special histological types 269 – e.g. definitionally low grade tumors, definitionally high grade tumors, non-gradable tumors, tumors 

with not-yet-established grading parameters. 

Clinical stage 272 – e.g. TNM/UICC staging system: stage I (A: low grade, small, superficial, and deep; B: low grade, large, superficial); 

stage II (A: low grade, large, deep; B: high grade, small, superficial, and deep; C: high grade, large, superficial); stage III (hig 

grade, large, deep), stage IV (any metastasis). 

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8 




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9 




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24 

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25 

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44 

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45 

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47 

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48 

Carr RA, Newman J, Antonakopulos GN, Parkinson MC. Lesions 

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49 

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50 

Nucci MR, Young RH, Fletcher CD. Cellular pseudosarcomatous 

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51 

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52 

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53 

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53a 

Zambrano E, Reyes-Mugica M, Franchi A, Rosai J. An osteoclast-rich 

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54 

Boyd AS, Wu H, Shyr Y. Monster cells in malignant melanoma. 


55 

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Table VI 

56 

Fisher C. Myofibroblastic malignancies. Adv Anat Pathol 

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57 

Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr 

NJ, Emory TS, et al. Gastrointestinal stromal tumors/smooth 

muscle tumors (GISTs) primary in the omentum and mesentery: 

clinicopathologic and immunohistochemical study of 26 cases. 


58 

Al-Nafussi A, Wong NA. Intra-abdominal spindle cell lesions: a 

review and practical aids to diagnosis. Histopathology 

2001;38:387-402. 

59 

Nakhleh RE, Wick MR, Rocamora A, Swanson PE, Dehner LP. 

Morphologic diversity in malignant melanomas. Am J Clin Pathol 

1990;93:731-40. 

59a 

Banerjee SS, Harris M. Morphological and immunophenotypic 

variations in malignant melanoma.Histopathology 2000;36:387-402. 

60 

Squillaci S, Cecchetti D, Tallarigo F, Pontieri E, Filardo AV. Myopericytoma-type, 

perivascular myoma located in the soft tissue 

of the foot: a case report and review of the literature. Pathologica 

2005;97:378-82. 

61 

Deyrup AT, Althof P, Zhou M, Morgan M, Solomon AR, Bridge 

JA, et al. Paraganglioma-like dermal melanocytic tumor: a unique 

entity distinct from cellular blue nevus, clear cell sarcoma, and 

cutaneous melanoma. Am J Surg Pathol 2004;28:1579-86. 

62 

Bridge JA, Fidler ME, Neff JR, Degenhardt J, Wang M, Walker 

C, et al. Adamantinoma-like Ewing's sarcoma: genomic confirmation, 

phenotypic drift. Am J Surg Pathol 1999;23:159-65. 

62a 

Folpe AL, Goldblum JR, Rubin BP, Shehata BM, Liu W, Dei Tos 

AP, et al. Morphologic and immunophenotypic diversity in Ewing 

family tumors: a study of 66 genetically confirmed cases. Am J 

Surg Pathol 2005;29:1025-33.


Table VII 

63 

Nappi O, Ritter JH, Pettinato G, Wick MR. Hemangiopericytoma: 

histopathological pattern or clinicopathologic entity? 

Semin Diagn Pathol 1995;12:221-32. 

64 

Gengler C, Guillou L. Solitary fibrous tumor and haemangiopericytoma: 

evolution of a concept. Histopathology 2006;48:63-74. 

65 

Orvieto E, Furlanetto A, Laurino L, Dei Tos AP. Myxoid and 

round cell liposarcoma: a spectrum of myxoid adipocytic neoplasia. 


66 

Schwartz RA, Dabski C, Dabska M. The Dabska tumor: a thirty 

year retrospect. Dermatology 2000;201:1-5. 

66a 

Urso C, Tinacci G. Angiomatoid cellular blue nevus: a variant of 

blue nevus with an angioma-like appearance. J Cutan Pathol 

2005;32:385-7. 

67 

Costa MJ, McGlothlen L, Pierce M, Munn R, Vogt PJ. Angiomatoid 

features in fibrohistiocytic sarcomas. Immunohistochemical, 

ultrastructural, and clinical distinction from vascular neoplasms. 

Arch Pathol Lab Med 1995;119:1065-71. 

67a 

von Hochstetter AR, Meyer VE, Grant JW, Honegger HP, 

Schreiber A. Epithelioid sarcoma mimicking angiosarcoma: the 

value of immunohistochemistry in the differential diagnosis. Virchows 

Arch A Pathol Anat Histopathol 1991;418:271-8. 

67b 

Fisher C. Epithelioid sarcoma of Enzinger. Adv Anat Pathol 

2006;13:114-21. 

Table VIII 

68 

Kazakov DV, Pitha J, Sima R, Vanecek T, Shelekhova K, Mukensnabl 

P, et al. Hybrid peripheral nerve sheath tumors: Schwannoma-perineurioma 

and neurofibroma-perineurioma. A report of 

three cases in extradigital locations. Ann Diagn Pathol 

2005;9:16-23. 

69 

Michal M, Kazakov DV, Belousova I, Bisceglia M, Zamecnik M, 

Mukensnabl P. A benign neoplasm with histopathological features 

of both schwannoma and retiform perineurioma (benign 

schwannoma-perineurioma): a report of six cases of a distinctive 

soft tissue tumor with a predilection for the fingers. Virchows 

Arch 2004;445:347-53. 

70 

Usta U, Turkmen E, Mizrak B, Yildiz D, Guzel Z. Spindle cell 

lipoma in an intramuscular lipoma. Pathol Int 2004;54:734-9. 

71 

Nascimento AG. Dedifferentiated liposarcoma. Semin Diagn 

Pathol 2001;18:263-6. 

72 

Elgar F, Goldblum JR. Well-differentiated liposarcoma of the 

retroperitoneum: a clinicopathologic analysis of 20 cases, with 

particular attention to the extent of low-grade dedifferentiation. 

Mod Pathol 1997;10:113-20. 

73 

Pool SE, Manieei F, Clark WH Jr, Harrist TJ. Dermal squamomelanocytic 

tumor: a unique biphenotypic neoplasm of uncertain 

biological potential. Hum Pathol 1999;30:525-9. 

74 

Erickson LA, Myers JL, Mihm MC, Markovic SN, Pittelkow 

MR. Malignant basomelanocytic tumor manifesting as metastatic 

melanoma. Am J Surg Pathol 2004;28:1393-6. 

Table IX 

75 

Saad AG, Mutema GK, Mutasim DF. Benign cutaneous epithelioid 

Schwannoma: case report and review of the literature. Am J 

Dermatopathol 2005;27:45-7. 

76 

Hornick JL, Fletcher CD. PEComa: what do we know so far? 


77 

Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, 

Faleri M, et al. Abdominopelvic sarcoma of perivascular epithelioid 

cells. Report of four cases in young women, one with tuberous 

sclerosis. Mod Pathol 2001;14:563-8. 

78 

Mentzel T, Reisshauer S, Rutten A, Hantschke M, Soares de 

Almeida LM, Kutzner H. Cutaneous clear cell myomelanocytic 

tumour: a new member of the growing family of perivascular epithelioid 

cell tumours (PEComas). Clinicopathological and immunohistochemical 

analysis of seven cases. Histopathology 

2005;46:498-504. 

79 

Nascimento AG, Keeney GL, Fletcher CD. Deciduoid peritoneal 

mesothelioma. An unusual phenotype affecting young females. 


80 

Shanks JH, Harris M, Banerjee SS, Eyden BP, Joglekar VM, et al. 

Mesotheliomas with deciduoid morphology: a morphologic spectrum 

and a variant not confined to young females. Am J Surg 

Pathol 2000;24:285-94. 

81 

Mourra N, de Chaisemartin C, Goubin-Versini I, Parc R, Flejou 

JF. Malignant deciduoid mesothelioma: a diagnostic challenge. 

Arch Pathol Lab Med 2005;129:403-6. 

82 

Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: 

clinicopathologic analysis of 14 cases of a rare epithelioid 

malignancy. Am J Surg Pathol 2004;28:1133-44. 

83 

d’Amore ES, Ninfo V. Clear cell tumors of the somatic soft tissues. 


84 

Lee AN, Stein SL, Cohen LM. Clear cell fibrous papule with 

NKI/C3 expression: clinical and histologic features in six cases. 


85 

Lazar AJ, Fletcher CD. Distinctive dermal clear cell mesenchymal 

neoplasm: clinicopathologic analysis of five cases. Am J 


85a 

Llombart-Bosch A, Pellin A, Carda C, Noguera R, Navarro S, 

Peydro-Olaya A. Soft tissue Ewing sarcoma--peripheral primitive 

neuroectodermal tumor with atypical clear cell pattern shows a 

new type of EWS-FEV fusion transcript. Diagn Mol Pathol 

2000;9:137-44. 

85b 

Plaza JA, Wakely PE Jr, Suster S. Lipoblastic nerve sheath tumors: 

report of a distinctive variant of neural soft tissue neoplasm 

with adipocytic differentiation. Am J Surg Pathol 

2006;30:337-44. 

85c 

Suster S, Fletcher CD. Gastrointestinal stromal tumors with 

prominent signet-ring cell features. Mod Pathol 1996;9:609-13. 

85d 

Rutten A, Huschka U, Requena C, Rodriguez-Peralto JL, Requena 

L. Primary cutaneous signet-ring cell melanoma: a clinicopathologic 

and immunohistochemical study of two cases. Am J 


85e 

Magro CM, Crowson AN, Mihm MC. Unusual variants of malignant 

melanoma. Mod Pathol 2006;19 Suppl 2:S41-70. 

85f 

Goldsmith JD, Pawel B, Goldblum JR, Pasha TL, Roberts S, Nelson 

P, et al. Detection and diagnostic utilization of placental alkaline 

phosphatase in muscular tissue and tumors with myogenic 

differentiation. Am J Surg Pathol 2002;26:1627-33. 

86 

Suster S. Tumors of the skin composed of large cells with abundant 

eosinophilic cytoplasm. Semin Diagn Pathol 1999;16:162-77. 

87 

d’Amore ES, Ninfo V. Tumors of the soft tissues composed of 

large eosinophilic cells. Semin Diagn Pathol 1999;16:178-89. 

88 

Lazar AJ, Fletcher CD. Primitive nonneural granular cell tumors 

of skin: clinicopathologic analysis of 13 cases. Am J Surg Pathol 

2005;29:927-34. 

89 

d’Amore ES, Ninfo V. Soft tissue small round cell tumors: morphological 

parameters. Semin Diagn Pathol 1996;13:184-203. 

90 

Dei Tos AP. Classification of pleomorphic sarcomas: where are 

we now? Histopathology 2006;48:51-62. 

91 

Oliveira AM, Nascimento AG. Pleomorphic liposarcoma. Semin 

Diagn Pathol 2001;18:274-85. 

92 

Oda Y, Miyajima K, Kawaguchi K, Tamiya S, Oshiro Y, Hachitanda 

Y, et al. Pleomorphic leiomyosarcoma: clinicopathologic 

and immunohistochemical study with special emphasis on its distinction 

from ordinary leiomyosarcoma and malignant fibrous 

histiocytoma. Am J Surg Pathol 2001;25:1030-8. 

93 

Sebenik M, Ricci A Jr, Di Pasquale B, Mody K, Pytel P, Jee KJ, 

et al. Undifferentiated intimal sarcoma of large systemic blood 

vessels: report of 14 cases with immunohistochemical profile and 

review of the literature. Am J Surg Pathol 2005;29:1184-93.

292 


Table X 

94 

Kinkor Z, Mukensnabl P, Michal M. Inflammatory myxohyaline 

tumor with massive emperipolesis. Pathol Res Pract 

2002;198:639-42. 

95 

Argani P, Facchetti F, Inghirami G, Rosai J. Lymphocyte-rich 

well-differentiated liposarcoma: report of nine cases. Am J Surg 

Pathol 1997;21:884-95. 

96 

Tarroch X, Vives P, Salas A, More J. Subcutaneous nodules in 

Whipple’s disease. J Cutan Pathol 2001;28:368-70. 

97 

Friedmann AC, Perera GK, Jayaprakasam A, Forgacs I, Salisbury 

JR, Creamer D. Whipple’s disease presenting with symmetrical 

panniculitis. Br J Dermatol 2004;15:907-11. 

Table XI 

98 

Zembowicz A, Mihm MC. Dermal dendritic melanocytic proliferations: 

an update. Histopathology 2004;45:433-51. 

99 

Carlson JA, Healy K, Slominski A, Mihm MC Jr. Melanocytic 

matricoma: a report of two cases of a new entity. Am J Dermatopathol 

1999;21:344-9. 

100 

Williams CM, Bozner P, Oliveri CV, Horenstein MG. Melanocytic 

matricoma: case confirmation of a recently described entity. J 

Cutan Pathol 2003;30:275-8. 

101 

Claessens N, Heymans O, Arrese JE, Garcia R, Oelbrandt B, Pierard 

GE. Cutaneous psammomatous melanotic schwannoma: nonrecurrence 

with surgical excision. Am J Clin Dermatol 

2003;4:799-802. 

102 

Swetter SM, Ecker PM, Johnson DL, Harvell JD. Primary dermal 

melanoma: a distinct subtype of melanoma. Arch Dermatol 

2004;140:99-103. 

103 

Zembowicz A, Carney JA, Mihm MC. Pigmented epithelioid 

melanocytoma: a low-grade melanocytic tumor with metastatic 

potential indistinguishable from animal-type melanoma and epithelioid 

blue nevus. Am J Surg Pathol 2004;28:31-40. 

104 

Crowson AN, Magro CM, Mihm MC Jr. Malignant melanoma 

with prominent pigment synthesis: “animal type” melanoma – a 

clinical and histological study of six cases with a consideration of 

other melanocytic neoplasms with prominent pigment synthesis. 

Hum Pathol 1999;30:543-50. 

105 

Carney JA, Ferreiro JA. The epithelioid blue nevus. A multicentric 

familial tumor with important associations, including cardiac 

myxoma and psammomatous melanotic schwannoma.Am J Surg 

Pathol 1996;20:259-72. 

106 

Moran CA, Albores-Saavedra J, Wenig BM, Mena H. Pigmented 

extraadrenal paragangliomas. A clinicopathologic and immunohistochemical 

study of five cases. Cancer 1997;79:398-402. 

Table XII 

107 

Blickenstaff RD, Roenigk RK, Peters MS, Goellner JR. Recurrent 

pyogenic granuloma with satellitosis. J Am Acad Dermatol 

1989;21:1241-4. 

108 

Blaauwgeers JL, Troost D, Dingemans KP, Taat CW, Van den 

Tweel JG. Multifocal rhabdomyoma of the neck. Report of a case 

studied by fine-needle aspiration, light and electron microscopy, 

histochemistry, and immunohistochemistry. Am J Surg Pathol 

1989;13:791-9. 

109 

Mahalingam M, Alter JN, Bhawan J. Multiple cellular neurothekeomas 

– a case report and review on the role of immunohistochemistry 

as a histologic adjunct. J Cutan Pathol 2006;33:51-6. 

Table XIII 

109a 

Agaram NP, Prakash S, Antonescu CR. Deep-seated plexiform 

schwannoma: a pathologic study of 16 cases and comparative 

analysis with the superficial variety. Am J Surg Pathol 

2005;29:1042-8. 

109b 

MacCollin M, Woodfin W, Kronn D, Short MP. Schwannomatosis: 

a clinical and pathologic study. Neurology 1996;46:1072-9 

(Comment in: Neurology 1997;48:787-8). 

109c 

Jacoby LB, Jones D, Davis K, Kronn D, Short MP, Gusella J, et 

al. Molecular analysis of the NF2 tumor-suppressor gene in 

schwannomatosis. Am J Hum Genet 1997;61:1293-302. 

109d 

Seppala MT, Sainio MA, Haltia MJ, Kinnunen JJ, Setala KH, 

Jaaskelainen JE. Multiple schwannomas: schwannomatosis or 

neurofibromatosis type 2? J Neurosurg. 1998;89:36-41 (Comment 

in: J Neurosurg 1999;91:351-2). 

110 

Woodruff JM, Scheithauer BW, Kurtkaya-Yapicier O, Raffel C, 

Amr SS, LaQuaglia MP, et al. Congenital and childhood plexiform 

(multinodular) cellular schwannoma: a troublesome mimic 

of malignant peripheral nerve sheath tumor. Am J Surg Pathol 

2003;27:1321-9. 

111 

Agaram NP, Prakash S, Antonescu CR. Deep-seated plexiform 

schwannoma: a pathologic study of 16 cases and comparative 

analysis with the superficial variety. Am J Surg Pathol 

2005;29:1042-8. 

112 

Shelekhova K, Kazakov DV, Michal M. Infiltrating retiform perineurioma: 

a case report. Ann Diagn Pathol 2005;9:293-4. 

113 

Clement PB, Young RH, Scully RE. Intravenous leiomyomatosis 

of the uterus. A clinicopathological analysis of 16 cases with unusual 

histologic features. Am J Surg Pathol 1988;12:932-45. 

114 

Calonje E, Mentzel T, Fletcher CD. Pseudomalignant perineurial 

invasion in cellular (‘infantile’) capillary haemangiomas. 


115 

Casadei GP, Scheithauer BW, Hirose T, Manfrini M, Van Houton 

C, Wood MB. Cellular schwannoma. A clinicopathologic, DNA 

flow cytometric, and proliferation marker study of 70 patients. 

Cancer 1995;75:1109-19. 

116 

Zelger BW, Calonje E, Sepp N, Fink FM, Zelger BG, Schmid 

KW. Monophasic cellular variant of infantile myofibromatosis. 

An unusual histopathologic pattern in two siblings. Am J Dermatopathol 

1995;17:131-8. 

Table XV 

117 

Brenn T, Fletcher CD. Postradiation vascular proliferations: an 

increasing problem. Histopathology 2006;48:106-14. 

Table XVI 

118 

Hollowood K, Fletcher CD. Soft tissue sarcomas that mimic benign 

lesions. Semin Diagn Pathol 1995;12:87-97. 

119 

Laurino L, Furlanetto A, Orvieto E, Dei Tos AP. Well-differentiated 

liposarcoma (atypical lipomatous tumors). Semin Diagn 

Pathol 2001;18:258-62. 

120 

Ritter JH, Humphrey PA, Wick MR. Malignant neoplasms capable 

of simulating inflammatory (myofibroblastic) pseudotumors 

and tumefactive fibroinflammatory lesions: pseudopseudotumors. 


121 

Nascimento AG. Spindle cell liposarcoma (Commentary). Adv 

Anat Pathol 1995;2:320-5. 

122 

Sneige N, Yaziji H, Mandavilli SR, Perez ER, Ordonez NG, 

Gown AM, et al. Low-grade (fibromatosis-like) spindle cell carcinoma 

of the breast. Am J Surg Pathol 2001;25:1009-16. 

Table XVII 

123 

Rosai J. Introduction: Dr. Lauren V. Ackerman and his man from 

Instanbul. Semin Diagn Pathol 2003;20:247-8. 

124 

Dehner LP. Juvenile xanthogranulomas in the first two decades of 

life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous 

manifestations. Am J Surg Pathol 2003;27:579-93. 

125 

Colby T. Visceral (pulmonary) juvenile xanthogranuloma. AMR 

International Slide Seminar # 39 (Case 4). November 2002 

(http://www.amr-seminar.org). 

126 

Kobayashi S, Yamadori I, Ohmori M, Akaeda T. Benign fibrous histiocytoma 

of the renal capsule. Acta Pathol Jpn 1992;42:217-20. 

127 

Sakakibara N, Seki T, Maru A, Koyanagi T. Benign fibrous histiocytoma 

of the kidney. J Urol 1989;142:1558-9. 

128 

Schick B, Kind M, Schwarzkopf G, Weber R, Draf W. Early 

childhood angiofibroma in an unusual site HNO 1997;45:1022-8.


128a 

Huang RY, Damrose EJ, Blackwell KE, Cohen AN, Calcaterra 

TC. Extranasopharyngeal angiofibroma. Int J Pediatr Otorhinolaryngol 

2000; 30:56:59-64. 

129 

Fisher C, Folpe AL, Hashimoto H, Weiss SW. Intra-abdominal 

synovial sarcoma: a clinicopathological study. Histopathology 

2004;45:245-53. 

130 

Ozolek JA, Carrau R, Barnes EL, Hunt JL. Nasal chondromesenchymal 

hamartoma in older children and adults: series and immunohistochemical 

analysis. Arch Pathol Lab Med 

2005;129:1444-50. 

131 

Montesco MC, Alaggio R, Ninfo V. Pediatric-type sarcomas in 

adult patients. Semin Diagn Pathol 2003;20:324-37. 

132 

Nascimento AF, Fletcher CD. Spindle cell rhabdomyosarcoma in 

adults. Am J Surg Pathol 2005;29:1106-13. 

133 

Huszar M, Fanburg JC, Dickersin GR, Kirshner JJ, Rosenberg 

AE. Retroperitoneal malignant meningioma. A light microscopic, 

immunohistochemical, and ultrastructural study. Am J Surg 

Pathol 1996;20:492-9. 

134 

Morrison C, Ramirez NC, Chan JK, Wakely P Jr. Endometrial 

stromal sarcoma of the retroperitoneum. Ann Diagn Pathol 

2002;6:312-8. 

135 

Bisceglia M, Attino V, Bacchi CE. Metastasizing “benign” fibrous 

histiocytoma of the skin: a report of two additional cases 

and review of the literature. Adv Anat Pathol 2006;13:89-96. 

136 

Kayser K, Zink S, Schenider T, Dienemann H, Andre S, Kaltner 

H, et al. Benign metastasizing leiomyoma of the uterus: documentation 

of clinical, immunohistochemicla and lectin-histochemical 

data of ten cases. Vircows Arch 2000;437:284-92. 

137 

Pramesh CS, Saklani AP, Pantvaidya GH, Heroor AA, Naresh 

KN, Sharma S, et al. Benign metastasizing meningioma. Jpn J 

Clin Oncol 2003;33:86-8. 

137a 

Navarro S, Noguera R, Pellin A, Lopez-Guerrero JA, Rosello- 

Sastre E, Cremades A, et al. Atypical pleomorphic extraosseous 

ewing tumor/peripheral primitive neuroectodermal tumor with 

unusual phenotypic/genotypic profile. Diagn Mol Pathol 

2002;11:9-15. 

138 

Carbone A, Gloghini A, Libra M, Gasparotto D, Navolanic PM, 

Spina M, et al. A spindle cell variant of diffuse large B-cell lymphoma 

possesses genotypic and phenotypic markers characteristic of a germinal 

center B-cell origin. Mod Pathol 2006;19:299-306. 

139 

Gu M, Antonescu CR, Guiter G, Huvos AG, Ladanyi M, Zakowski 

MF. Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence 

in 50 cases confirmed by molecular diagnostic studies. Am J 

Surg Pathol 2000;24:410-6. 

139a 

Collini P, Sampietro G, Bertulli R, Casali PG, Luksch R, Mezzelani 

A, et al. Cytokeratin immunoreactivity in 41 cases of ES/PNET confirmed 

by molecular diagnostic studies. Am J Surg Pathol 

2001;25:273-4. 

139b 

Schuetz AN, Rubin BP, Goldblum JR, Shehata B, Weiss SW, Liu 

W, et al. Intercellular junctions in Ewing sarcoma/primitive neuroectodermal 

tumor: additional evidence of epithelial differentiation. 

Mod Pathol 2005;18:1403-10. 

140 

Zarbo RJ, Gown AM, Nagle RB, Visscher DW, Crissman JD. 

Anomalous cytokeratin expression in malignant melanoma: oneand 

two-dimensional western blot analysis and immunohistochemical 

survey of 100 melanomas. Mod Pathol 1990;3:494-501. 

Table XVIII 

141 

Hurt MA, Santa Cruz DJ. Nodular-cystic fat necrosis. A reevaluation 

of the so-called mobile encapsulated lipoma. J Am Acad 

Dermatol 1989;21:493-8. 

141a 

Bisceglia M, Vigilante E, Ben-Dor D. Neural lipofibromatous 

hamartoma: a report of two cases and review of the literature. 

Adv Anat Pathol, in press 

Table XX 

142 

Lattes R, Bigotti G. Lipoblastic meningioma: “vacuolated meningioma”. 

Hum Pathol 1991;22:164-71. 

143 

Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic 

study of 30 cases. Hum Pathol 1982;13:210-8. 

144 

Davies PE, Davis GJ, Dodd T, Selva D. Orbital lipomatous haemangiopericytoma: 

an unusual variant. Clin Experiment Ophthalmol 

2002;30:281-3. 

Table XXI 

145 

Bennett S, Defoor W, Minevich E. Primary extrarenal nephrogenic 

rest. J Urol 2002;168:1529. 

146 

Bisceglia M, Carosi I, Castelvetere M, Murgo R. Multiple 

Fordyce-type angiokeratomas of the scrotum. A iatrogenic case. 

Pathologica 1998;90:46-50. 

147 

Popek EJ, Montgomery EA, Fourcroy JL. Fibrous hamartoma of 

infancy in the genital region: findings in 15 cases. J Urol 

1994;152:990-3. 

148 

Fisher C, Bisceglia M. Solitary fibrous tumour of the spermatic 

cord. Br J Urol 1994;74:798-99. 

149 

Churg A. Paratesticular mesothelial proliferations. Semin Diagn 

Pathol 2003;20:272-8. 

Table XXII 

150 

Nucci MR, Fletcher CD. Vulvovaginal soft tissue tumours: update 

and review. Histopathology 2000;36:97-108. 

151 

Vargas SO, Kozakewich HP, Boyd TK, Ecklund K, Fishman SJ, 

Laufer MR, et al. Childhood asymmetric labium majus enlargement: 

mimicking a neoplasm. Am J Surg Pathol 2005;29:1007-16. 

152 

Iwasa Y, Fletcher CD. Distinctive prepubertal vulval fibroma: a 

hitherto unrecognized mesenchymal tumor of prepubertal girls: 

analysis of 11 cases. Am J Surg Pathol 2004;28:1601-8. 

153 

Novick NL. Angiokeratoma vulvae. J Am Acad Deramtol 

1985;12:561-3. 

154 

Michal M, Rokyta Z, Mejchar B, Pelikán K, Kummel M, Mukensnabl 

P. Prolapse of Fallopian tube after hysterectomy associated 

with exuberant angiomyofibroblastic stroma response. A diagnostic 

pitfall. Virchows Arch 2000;437:436-9. 

155 

Varnholt H, Otis CN, Nucci MR, Johari VP. Fallopian tube prolapse 

mimicking aggressive angiomyxoma. Int J Gynecol Pathol 

2005;24:292-4. 

156 

Branton PA, Tavassoli FA. Spindle cell epithelioma, the so-called 

mixed tumor of the vagina. A clinicopathologic, immunohistochemical, 

and ultrastructural analysis of 28 cases. Am J Surg 

Pathol 1993;17:509-15. 

157 

Skelton H, Smith KJ. Spindle cell epithelioma of the vagina 

shows immunohistochemical staining supporting its origin from a 

primitive/progenitor cell population. Arch Pathol Lab Med 

2001;125:547-50. 

158 

Nucci MR, Fletcher CD. Liposarcoma (atypical lipomatous tumors) 

of the vulva: a clinicopathologic study of six cases. Int J 

Gynecol Pathol 1998;17:17-23. 

158a 

Bakotic BW, Cabello-Inchausti B, Willis IH, Suster S. Clear-cell 

epithelioid leiomyoma of the round ligament. Mod Pathol 

1999;12:912-8. 

159 

Hassan A, Bennet A, Bhalla S, Ylagan LR, Mutch D, Dehner LP. 

Paraganglioma of the vagina: report of a case, including immunohistochemical 

and ultrastructural findings. Int J Gynecol 

Pathol 2003;22:404-6. 

160 

Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. 

Perivascular epithelioid cell neoplasms of soft tissue and gynecologic 

origin: a clinicopathologic study of 26 cases and review 

of the literature. Am J Surg Pathol 2005;29:1558-75. 

161 

Banerjee SS, Coyne JD, Menasce LP, Lobo CJ, Hirsch PJ. Diagnostic 

lessons of mucosal melanoma with osteocartilaginous differentiation. 


Table XXIII 

162 

Gunia S, Behrens MH, Stosiek P. Mycobacterial spindle cell

294 


pseudotumor (MSP) of the nasal septum clinically mimicking Kaposi’s 

sarcoma: case report. Rhinology 2005;43:70-1. 

163 

Kindblom LG, Angervall L. Nasal polyps with atypical stroma 

cells: a pseudosarcomatous lesion. A light and electron-microscopic 

and immunohistochemical investigation with implications 

on the type and nature of the mesenchymal cells. Acta Pathol Microbiol 

Immunol Scand 1984;92:65-72. 

164 

Nakayama M, Wenig BM, Heffner DK. Atypical stromal cells in 

inflammatory nasal polyps: immunohistochemical and ultrastructural 

analysis in defining histogenesis. Laryngoscope 

1995;105:127-34. 

165 

Pai SA, Naresh KN, Masih K, Ramarao C, Borges AM. Teratocarcinosarcoma 

of the paranasal sinuses: a clinicopathologic 

and immunohistochemical study. Hum Pathol 1998;29:718-22. 

166 

Kleinschmidt-DeMasters BK, Pflaumer SM, Mulgrew TD, Lillehei 

KO. Sinonasal teratocarcinosarcoma (“mixed olfactory neuroblastoma-craniopharyngioma”) 

presenting with syndrome of 

inappropriate secretion of antidiuretic hormone. Clin Neuropathol 

2000;19:63-9. 

167 

Endo H, Hirose T, Kuwamura KI, Sano T. Case report: Sinonasal 

teratocarcinosarcoma. Pathol Int 2001;51:107-12. 

168 

Hicks DL, Moe KS. Nasal solitary fibrous tumor arising from the 

anterior cranial fossa. Skull Base 2004;14:203-7. 

169 

Fukunaga M, Ushigome S, Nomura K, Ishikawa E. Solitary fibrous 

tumor of the nasal cavity and orbit. Pathol Int 1995;45:952-7. 

170 

Eichhorn JH, Dickersin GR, Bhan AK, Goodman ML. Sinonasal 

hemangiopericytoma. A reassessment with electron microscopy, 

immunohistochemistry, and long-term follow-up. Am J Surg 

Pathol 1990;14:856-66. 

171 

Thompson LD, Miettinen M, Wenig BM. Sinonasal-type hemangiopericytoma: 

a clinicopathologic and immunophenotypic 

analysis of 104 cases showing perivascular myoid differentiation. 


172 

Huang HY, Antonescu CR. Sinonasal smooth muscle cell tumors: 

a clinicopathologic and immunohistochemical analysis of 12 cases 

with emphasis on the low-grade end of the spectrum. Arch 

Pathol Lab Med 2003;127:297-304. 

173 

Bel Haj Salah M, Mekni A, Nouira K, Kharrat S, Bellil K, Bellil 

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174 

Kuruvilla A, Wenig BM, Humphrey DM, Heffner DK. 

Leiomyosarcoma of the sinonasal tract. A clinicopathologic study 

of nine cases. Arch Otolaryngol Head Neck Surg 1990;116:1278-86. 

175 

Banerjee SS, Eyden B, Trenholm PW, Sheikh MY, Wakamatsu 

K, Ancans J, et al. Monotypic angiomyolipoma of the nasal cavity: 

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2001;9:309-15. 

176 

Begin LR, Rochon L, Frenkiel S. Spindle cell myoepithelioma of 

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177 

Iezzoni JC, Mills SE. “Undifferentiated” small round cell tumors 

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178 

Magro G, Di Benedetto A, Sanges G, Scalisi F, Alaggio R. Rhabdomyomatous 

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2005;446:346-7. 

179 

Yoshihara T, Oda A, Takahashi Y, Ishii T, Yaku Y. Fibrous hamartoma 

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180 

Schlosnagle DC, Kratochvil FJ, Weathers DR, McConnel FM, 

Campbell WG Jr. Intraoral multifocal adult rhabdomyoma. Report 

of a case and review of the literature. Arch Pathol Lab Med 

1983;107:638-42. 

181 

Said-Al-Naief N, Zahurullah FR, Sciubba JJ. Oral spindle cell 

lipoma. Ann Diagn Pathol 2001;5:207-15. 

182 

Ide F, Shimoyama T, Horie N. Pleomorphic hyalinizing angiectactic 

tumor of the buccal mucosa. J Oral Pathol Med 

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183 

Ramaraj PN, Shah SP. Peripheral myxoma of maxilla. A case report. 

Indian J Dent Res 2003;14:67-9. 

184 

Lukinmaa PL, Hietanen J, Warfvinge G, Sane J, Tuominen S, 

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of three cases. J Oral Pathol Med 2000;29:186-92. 

185 

Wu SL, Vang R, Clubb FJ Jr, Connelly JH. Solitary fibrous tumor 

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studies. Ann Diagn Pathol 2002;6:168-71. 

186 

Chi AC, Weathers DR, Folpe AL, Dunlap DT, Rasenberger K, 

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Med Oral Pathol Oral Radiol Endod 2005;100:717-24. 

187 

Fisher C. Clear cell sarcoma (of tendon sheath) arising in palate. 

AMR International Slide Seminar #39 (Case 10). November 2002 

(http://www.amr-seminar.org) 

188 

Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers 

EL. Lingual alveolar soft part sarcoma; 14 cases: novel clinical 

and morphological observations. Histopathology 2004;45:526-37. 

189 

James JA, Bali NS, Sloan P, Shanks JH. Low-grade malignant 

Triton tumor of the oral cavity: a case report. Oral Surg Oral Med 

Oral Pathol Oral Radiol Endod 2003;95:699-704. 

190 

Massarelli G, Tanda F, Salis B. Synovial sarcoma of the soft 

palate: report of a case. Hum Pathol 1978;9:341-5. 

191 

Sakai Y, Matsukuma S. CD34+ stromal cells and hyalinized vascular 

changes in the anal fibroepithelial polyps. Histopathology 

2002;41:230-5. 

192 

Miettinen M, Furlong M, Sarlomo-Rikala M, Burke A, Sobin LH, 

et al. Gastrointestinal stromal tumors, intramural leiomyomas, 

and leiomyosarcomas in the rectum and anus: a clinicopathologic, 

immunohistochemical, and molecular genetic study of 144 

cases. Am J Surg Pathol 2001;25:1121-33. 

192a 

Tworek JA, Goldblum JR, Weiss SW, Greenson JK, Appelman 

HD. Stromal tumors of the anorectum: a clinicopathologic study 

of 22 cases. Am J Surg Pathol 1999;23:946-54 

192b 

Rubin BP. Gastrointestinal stromal tumours: an update. 


Table XXV 

193 

Cook PD, Czerniak B, Chan JK, Mackay B, Ordonez NG, Ayala 

AG, et al. Nodular spindle-cell vascular transformation of lymph 

nodes. A benign process occurring predominantly in retroperitoneal 

lymph nodes draining carcinomas that can simulate Kaposi's 

sarcoma or metastatic tumor. Am J Surg Pathol 

1995;19:1010-20. 

194 

Bisceglia M, Nirchio V, Carosi I, Cappucci U, Decata A, 

Paragone T, et al. Tumor and tumor-like benign mesenchymal lesions 

of the breast. Pathologica 1995;87:20-41. 

195 

Magro G, Michal M, Bisceglia M. Benign spindle cell tumors of 

the mammary stroma: diagnostic criteria, classification, and histogenesis. 

Pathol Res Pract 2001;197:453-66. 

196 

Brogi E. Benign and malignant spindle cell lesions of the breast. 


197 

Shin SJ, Scamman W, Gopalan A, Rosen PP. Mammary presentation 

of adult-type “juvenile” xanthogranuloma. Am J Surg Pathol 

2005;29:827-31. 

198 

Leibl S, Moinfar F. Mammary NOS-yype sarcoma with CD10 expression: 

a rare entity with features of myoepithelial differentiation. 


199 

Kurian KM, Al-Nafussi A. Sarcomatoid/metaplastic carcinoma 

of the breast: a clinicopathological study of 12 cases. 


199a 

Carter MR, Hornick JL, Lester S, Fletcher CD. Spindle cell (sar-


comatoid) carcinoma of the breast: a clinicopathologic and immunohistochemical 

analysis of 29 cases. Am J Surg Pathol 

2006;30:300-9 

Table XXVI 

200 

Vargas SO. Fibrous umbilical polyp: a distinct fasciitis-like proliferation 

of early childhood with a marked male predominance. 


200a 

Alaggio R, Ninfo V, Rosolen A, Coffin CM. Primitive myxoid 

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200b 

Reis-Filho JS, Milanezi F, Ferro J, Schmitt FC. Pediatric pigmented 

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diagnosis. Pathol Res Pract 2002;198:621-6. 

201 

Casanova M, Ferrari A, Collini P, Bisogno G, Alaggio R, Cecchetto 

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a report from the Italian Soft Tissue Sarcoma Committee. Cancer 

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202 

Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, 

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Oncol 2005;23:8422-30. 

203 

Ferrari A, Casanova M, Bisogno G, Cecchetto G, Meazza C, Gandola 

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Cooperative Group. Med Pediatr Oncol 2002;39:109-14. 

204 

Ferrari A, Casanova M, Bisogno G, Mattke A, Meazza C, Gandola 

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pediatric patients: a report from the Italian and German Soft Tissue 

Sarcoma Cooperative Group. Cancer 2002;94:3269-76. 

205 

Bisogno G, Sotti G, Nowicki Y, Ferrari A, Garaventa A, Zanetti I, 

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Table XXVII 

206 

Winkelmann RK, Grado GL, Quimby SR, Connolly SM. Pseudosclerodermatous 

panniculitis after irradiation: an unusual 

complication of megavoltage treatment of breast carcinoma. 

Mayo Clin Proc 1993;68:122-7. 

207 

Kondi-Pafiti A, Grapsa D, Kontogianni-Katsarou K, Papadias K, 

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208 

Billings SD, McKenney JK, Folpe AL, Hardacre MC, Weiss SW. 

Cutaneous angiosarcoma following breast-conserving surgery 

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2004;28:781-8. 

209 

Essary LR, Wick MR. Cutaneous calciphylaxis. An underrecognized 

clinicopathologic entity. Am J Clin Pathol 2000;113:280-7. 

210 

McGregor DH, Mowry M, Cherian R, McAnaw M, Poole E. Nonfamilial 

tumoral calcinosis associated with chronic renal failure 

and secondary hyperparathyroidism: report of two cases with 

clinicopathological, immunohistochemical, and electron microscopic 

findings. Hum Pathol 1995;26:607-13. 

211 

Bisceglia M, Grossi GB, Panniello G, Bertani T, Cenacchi G, 

Pasquinelli G. Amyloidosis. Clinico-pathological picture. Part I: 

Nosography. Pathologica 1997;89:329-45. 

212 

Bisceglia M, Grossi GB, Panniello G, Bertani T, Cenacchi G, 

Pasquinelli G. Amyloidosis. Clinico-pathological profile. Part II: 

Diagnosis. Pathologica 1998;90:57-78. 

213 

Young RH, Clement PB, McCaughey WT. Solitary fibrous tumors (‘fibrous 

mesotheliomas’) of the peritoneum. A report of three cases and 

a review of the literature. Arch Pathol Lab Med 1990;114:493-5. 

214 

Carney JA, Stratakis CA. Familial paraganglioma and gastric 

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Am J Med Genet 2002;108:132-9. 

214a 

Perry CG, Young WF Jr, McWhinney SR, Bei T, Stergiopoulos S, 

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215 

Deyrup AT, Lee VK, Hill CE, Cheuk W, Toh HC, Kesavan S, et 

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a clinicopathologic and molecular analysis of 29 tumors from 19 

patients. Am J Surg Pathol 2006;30:75-82. 

215 

Burke AP, Virmani R. Cardiac myxoma. A clinicopathologic 

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216 

Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic 

study. Cancer 1993;71:1761-73. 

Table XXVIII 

217 

Vincent A, Farley M, Chan E, James WD. Birt-Hogg-Dube syndrome: 

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firm facial papules. J Am Acad Dermatol 2003;49:698-705. 

217a 

Bisceglia M, Carosi I, Simeone A. Tuberous sclerosis complex 

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218 

Stewart NJ, Pritchard DJ, Nascimento AG, Kang YK. Lymphangiosarcoma 

following mastectomy. Clin Orthop Relat Res 

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218a 

Bisceglia M, Attino V, Dimitri L, Armillotta M, Centola A, D’Angelo 

V. Le manifestazioni cutanee ed extracutanee della neurofibromatosi. 

G Ital Dermatol Venereol 2003;138:86-91. 

219 

Gorlin RJ. Nevoid basal cell carcinoma (Gorlin) syndrome. Genet 

Med 2004;6:530-9. 

220 

Watson J, Depasquale K, Ghaderi M, Zwillenberg S. Nevoid 

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221 

Lee HY, Yoon CS, Sevenet N, Rajalingam V, Delattre O, Walford 

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222 

Olivier M, Goldgar DE, Sodha N, Ohgaki H, Kleihues P, Hainaut 

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2003;63:6643-50. 

Table XXIX 

223 

Ibrahim AE, Myles L, Lang DA, Ellison DW. Case of the month: 

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1998;8:817-8. 

224 

Horenstein MG, Manci EA, Walker AB, Dehner LP. Lumbosacral 

ectopic nephrogenic rest unassociated with spinal dysraphism. 


225 

Ober WB. Emil Zuckerkandl and his delightful little organ. 

Pathol Annu 1983;18:103-19. 

226 

Blecha M, Galanopolous C, Dharkar D, Salti G. Massive organ of 

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2005;201:480-1. 

227 

Bee D, Howard P. The carotid body: a review of its anatomy, 

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228 

Wang ZY, Bisgard GE. Chronic hypoxia-induced morphological 

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229 

Pantanowitz L, Balogh K. Significance of the juxtaoral organ (of 

Chievitz). Head Neck 2003;25:400-5. 

230 

Ide F, Mishima K, Saito I. Juxtaoral organ of Chievitz presenting 

clinically as a tumour. J Clin Pathol 2003;56:789-90. 

231 

Ide F, Mishima K, Saito I. Pacinian corpuscle in the juxtaoral organ 

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232 

Fraitag S, Gherardi R, Wechsler J. Hyperplastic pacinian corpuscles: 

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296 


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233 

Rinaldi P, Andreini A, Ercolani C, Bernardi L, Bernardi S. Digital 

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234 

Drut R, Galliani C. Thymic tissue in the skin: a clue to the diagnosis 

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Int J Surg Pathol 2003;11:25-8. 

Table XXX 

235 

Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley 

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236 

Weiss SW, Goldblum J. Benign soft tissue tumors and pseudotumors 

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237 

Fletcher CDM, Unni KK, Mertens F. World Health Organization 

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Soft Tissue and Bone. Lyon: IARC Press 2002. 

238 

Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas 

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in 28 patients. Am J Surg Pathol 1988;12:519-30. 

239 

Siassi RM, Papadopoulos T, Matzel KE. Metastasizing aggressive 

angiomyxoma. N Engl J Med 1999;341:1772. 

240 

Blandamura S, Cruz J, Faure Vergara L, Machado Puerto I, Ninfo 

V. Aggressive angiomyxoma: a second case of metastasis with 

patient’s death. Hum Pathol 2003;34:1072-4. 

241 

Nielsen GP, Young RH, Dickersin GR, Rosenberg AE. Angiomyofibroblastoma 

of the vulva with sarcomatous transformation 

(“angiomyofibrosarcoma”). Am J Surg Pathol 1997;21:1104-8. 

242 

Billings SD, Folpe AL, Weiss SW. Do leiomyomas of deep soft 

tissue exist? An analysis of highly differentiated smooth muscle 

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Surg Pathol 2001;25:1134-42. 

243 

Miettinen M, Fetsch JF. Evaluation of biological potential of 

smooth muscle tumours. Histopathology 2006;48:97-105. 

243a 

Hornick JL, Fletcher CD. Criteria for malignancy in nonvisceral 

smooth muscle tumors. Ann Diagn Pathol 2003;7:60-6. 

244 

McMenamin ME, Fletcher CD. Malignant myopericytoma: expanding 

the spectrum of tumours with myopericytic differentiation. 


245 

O’Hara CD, Nascimento AG. Endothelial lesions of soft tissues: 

a review of reactive and neoplastic entities with emphasis on lowgrade 

malignant (“borderline”) vascular tumors. Adv Anat 

Pathol 2003;10:69-87. 

246 

Hunt SJ, Santa Cruz DJ. Vascular tumors of the skin: a selective 

review. Semin Diagn Pathol 2004;21:166-218. 

247 

White W, Shiu MH, Rosenblum MK, Erlandson RA, Woodruff 

JM. Cellular schwannoma. A clinicopathologic study of 57 patients 

and 58 tumors. Cancer 1990;66:1266-75. 

248 

Hornick JL, Fletcher CD. Soft tissue perineurioma: clinicopathologic 

analysis of 81 cases including those with atypical histologic 

features. Am J Surg Pathol 2005;29:845-58. 

249 

Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral 

nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, 

and ultrastructural study of seven cases. Am 

J Surg Pathol 1998;22:1368-78. 

250 

Zamecnik M, Michal M. Malignant peripheral nerve sheath tumor 

with perineurial cell differentiation (malignant perineurioma). 

Pathol Int 1999;49:69-73. 

251 

Rosenberg AS, Langee CL, Stevens GL, Morgan MB. Malignant 

peripheral nerve sheath tumor with perineurial differentiation: 

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252 

Michal M, Neubauer L, Fakan F. Carcinoma arising in ectopic 

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253 

Guillou L, Gebhard S, Salmeron M, et al. Metastasizing fibrous 

histiocytoma of the skin: a clinicopathologic and immunohistochemical 

analysis of three cases. Mod Pathol 2000;13:654-60. 

254 

Folpe AL, Weiss SW. Pleomorphic hyalinizing angiectatic tumor: 

analysis of 41 cases supporting evolution from a distinctive precursor 

lesion. Am J Surg Pathol 2004;28:1417-25. 

255 

Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical 

and malignant glomus tumors: analysis of 52 cases, with a proposal 

for the reclassification of glomus tumors. Am J Surg Pathol 

2001;25:1-12. 

256 

Oliveira AM, Dei Tos AP, Fletcher CD, Nascimento AG. Primary 

giant cell tumor of soft tissues: a study of 22 cases. Am J Surg 

Pathol 2000;24:248-56. 

257 

Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade 

fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis 

of 19 cases with a unique observation in the pediatric population. 


258 

Bisceglia M, Vairo M, Calonje E, Fletcher CD. Pigmented fibrosarcomatous 

dermatofibrosarcoma protuberans (Bednar tumor). 

3 case reports, analogy with the “conventional” type and 

review of the literature. Pathologica 1997;89:264-73. 

259 

Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD. Fibrosarcomatous 

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260 

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261 

Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, Shia 

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262 

Antonescu CR, Argani P, Erlandson RA, Healey JH, Ladanyi M, 

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263 

Suster S. Malignant glomus tumor with systemic metastases. 

AMR International Slide Seminar #14 (Case 11). March 1994 

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264 

Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. 

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265 

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266 

Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel 

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267 

Coindre JM, Trojani M, Contesso G, David M, Rouesse J, Bui 

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268 

Guillou L, Coindre JM, Bonichon F, Nguyen BB, Terrier P, Collin 

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269 

Association of Directors of Anatomic and Surgical Pathology. 

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270 

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271 

Deyrup AT, Weiss SW. Grading of soft tissue sarcomas: the challenge 

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272 

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Index 

This Index lists alphabetically only those entities which appeared in the several preceding Excerpta. 

In parenthesis is the reference to the particular Excerpta published in Pathologica which describes the entity in detail. 

Acquired elastotic hemangioma (2005;97:10-22 / IV-1). 

Acral inflammatory myxohyaline tumor with Reed-Sternberg-like cells (2004;96:436-63 / II-8) 

Amyloid tumor (2006;98:187-208 / IX-3) 

Angiomyofibroblastoma of vulva (2004;96:436-63 / II-4) 

Atypical decubital fibroplasia (2005;97:394-408 / VIII-4) 

Atypical fibroxanthoma - non-pleomorphic spindle-cell (2004;96:121-42; I-2) 

Atypical fibroxanthoma - aneurysmal (pigmented) variants (2004;96:121-42; I-2) 

Bacillary epithelioid angiomatosis (2005;97:394-408 / VIII-6) 

Bednar tumor - fibrosarcomatous (2004;96:121-42 / I-1) 

Bizarre parosteal osteochondromatous proliferation (2006;98:187-208 / IX-1) 

Calcifying fibrous (pseudo)tumor (2004;96:436-63 / II-7) 

Calcium pyrophosphate dehydrate crystals deposition disease (tumoral form) (2006;98:187-208 / IX-4) 

Castleman’s disease of the subcutis and skeletal muscle (2005;97:141-57 / VI-5) 

Chondroid lipoma (2004;96:481-95 / III-2) 

CPPD (calcium pyrophosphate dihydrate) crystals deposition disease (2006;98:187-208 / IX-2) 

Dabska tumor arising on lymphangioma (2005;97:10-22 / IV-4) 

Dabska’s parachordoma (2005;97:343-60 / VII-2) 

Dendritic cell neurofibroma with pseudorosettes (2005;97:92-114 / V-3) 

Dendritic fibromyxolipoma (2004;96:481-95 / III-3) 

Dermatofibrosarcoma protuberans – sarcomatous, classic type (2004;96:121-42 / I-1) 

Dermatofibrosarcoma protuberans – sarcomatous, pigmented variant (2004;96:121-42 / I-1) 

Dermatomyofibroma (2004;96:436-63 / II-1) 

Desmoplastic fibroblastoma (2004;96:121-42 / I-6) 

Ectopic hamartomatous thymoma (2005;97:394-408 / VIII-1) 

Epithelial sheath neuroma (2005;97:92-114 / V-4) 

Epithelioid hemangioendothelioma of soft tissue and skin (2005;97:10-22 / IV-2) 

Epithelioid leiomyosarcoma (2004;96:481-95 / III-6) 

Epithelioid liposarcoma (2004;96:481-95 / III-4) 

Epithelioid psammomatous melanotic schwannoma (2005;97:92-114 / V-1) 

Ewing’s sarcoma of the skin (2005;97:92-114 / V-6) 

Extraneural perineurioma (2005;97:92-114 / V-5B) 

Extraneuraxial hemangioblastoma of soft tissue and skin (2005;97:10-22 / IV-3) 

Extra-renal malignant rhabdoid tumour (2005;97:141-57 / VI-3) 

Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation (2005;97:141-57 / VI-1) 

Fibroosseous pseudotumor of the digits (2006;98:187-208 / IX-1) 

Fibrosarcomatous dermatofibrosarcoma protuberans, classic type (2004;96:121-42 / I-1) 

Fibrosarcomatous Bednar tumor (dermatofibrosarcoma protuberans, pigmented type) (2004;96:121-42 / I-1) 

Florid reactive periostitis of the tubular bones of the hands and feet (2006;98:187-208 / IX-2) 

Follicular dendritic cell sarcoma (2005;97:141-57 / VI-4) 

Glomus coccygeum (2006;98:187-208 / IX-4)) 

Granular cell leiomyosarcoma (2004;96:481-95 / III-7) 

Hamartoma of the scalp with ectopic meningothelial elements (2005;97:394-408 / VIII-2) 

Hemosiderotic fibrohistiocytic lipomatous lesion (2004;96:121-42 / I-4) 

Hyaline cell-rich chondroid syringoma (2005;97:343-60 / VII-1C) 

Hyalinizing spindle cell tumor with giant rosettes (2004;96:436-63 / II-11) 

Inflammatory myofibroblastic tumor (2004;96:436-63 / II-6) 

Inflammatory pseudotumor (2004;96:436-63 / II-6) 

Inflammatory myxo-hyaline tumor (2004;96:436-63 / II-8) 

Intranodal hemorrhagic spindle cell tumor with “amianthoid” fibers (2004;96:436-63 / II-2) 

Intraneural perineurioma (2005;97:92-114 / V-5A) 

Ischemic fasciitis (2005;97:394-408 / VIII-4)

298 


Juxtarticular myxoma (2004;96:436-63 / II-9) 

Lipoleiomyosarcoma (2004;96:481-95 / III-5) 

Lipomatous hemangiopericytoma (2004;96:436-63 / II-5) 

Localized hypertrophic (mono)neuropathy (2005;97:92-114 / V-5A) 

Low grade fibromyxoid sarcoma (2004;96:436-63 / II-11) 

Mixed tumor (2005;97:343-60 / VII-1A) 

Myoepithelioma (2005;97:343-60 / VII-1B) 

Myofibrosarcoma (2004;96:436-63 / II-3) 

Myxoinflammatory fibroblastic sarcoma (2004;96:436-63 / II-8) 

Nephrogenic fibrosing dermopathy (2005;97:394-408 / VIII-5) 

Neural fibrolipomatous hamartoma (2005;97:394-408 / VIII-3) 

Nora’s lesion (2006;98:187-208 / IX-1) 

Nuchal-type fibroma (2004;96:121-42 / I-5) 

Oncocytoma (2005;97:343-60 / VII-3) 

Ossifying fibromyxoid tumor (2004;96:436-63 / II-10) 

Palisaded myofibroblastoma (2004;96:436-63 / II-2) 

Perineuriomas - intraneural and extraneural forms (2005;97:92-114 / V-5) 

Pigmented neurofibroma (2005;97:92-114 / V-2) 

Phosphaturic mesenchymal tumor (2004;96:481-95 / III-1) 

Pleomorphic hyalinizing angiectatic tumor (2004; 96:121-42 / I-8) 

Plexiform fibrohistiocytic tumor (2004;96:121-42 / I-3) 

Plexiform xanthomatous tumor (2005;97:141-57 / VI-7) 

Post-implant parathyromatosis (in heterotopic locations) (2006;98:187-208 / IX-5) 

Proximal-type epithelioid sarcoma (2005;97:141-57 / VI-2) 

Rosai-Dorfman disease of soft tissue (2005;97:141-57 / VI-6) 

Rudimentary meningocele (2005;97:394-408 / VIII-2) 

Sclerosing epithelioid fibrosarcoma (2004; 96:121-42 / I-9). 

Soft tissue perineurioma (2005;97:92-114 / V-5B) 

Solitary fibrous tumor (2004;96:121-42 / I-7) 

Superficial angiomyxoma (2004;96:436-63 / II-9) 

Tophaceous pseudogout (2006;98:187-208 / IX-2) 

Tumoral calcium pyrophosphate dihydrate crystals deposition disease (CPPDCDD) (2006;98:187-208 / IX-2)

Tumoral, quasitumoral and pseudotumoral lesions of the ... - Siapec

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