Invasive breast carcinoma - IARC

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A C Fig. 6.17 Sarcoma botryoides. A Polypoid masses of tumour are covered by squamous epithelium. B A subepithelial cambium layer overlies oedematous tissue. C Higher magnification of the cellular subepithelial cambium layer. D The positive immunostain for desmin confirms the myoblastic differentiation. Note the nuclear pleomorphism. The clinical setting, the characteristic low power appearance, the absence of a cambium layer and striated cells and a typically low mitotic index establish the c o r rect diagnosis of a fibro e p i t h e l i a l polyp {2055,2067,2141}. Genetic susceptibility One instance of sarcoma botryoides has been reported in a child with multiple congenital abnormalities and bilateral nephroblastomas suggesting a possible genetic defect {1965}. Prognosis and predictive factors The prognosis of sarcoma botryoides in the past was poor, but an 85% 3-year survival rate has recently been achieved with wide local excision and combination chemotherapy. Second malignancies in long-term survivors of vaginal embryonal r h a b d o m y o s a rcoma have not been reported to date. Leiomyosarcoma Definition A malignant tumour composed of smooth muscle cells. Epidemiology Although leiomyosarcoma is the most common vaginal sarcoma in the adult and the second most common vaginal B D sarcoma, only approximately 50 cases have been reported {599}. They accounted for only 5 of 60 cases in the only large series of vaginal smooth muscle tumours {2879}. Macroscopy M a c ro s c o p i c a l l y, they are sometimes multilobulated and form masses from 3-5 cm. The sectioned surface has a pinkgrey, "fish-flesh" appearance with scatt e red foci of haemorrhage, myxoid change or necrosis. The overlying mucosa may be ulcerated. Histopathology Histologically, they are identical to their counterparts elsewhere. It may be difficult to predict the behaviour of some smooth muscle tumours. Currently, it is recommended that vaginal smooth muscle tumours that are larger than 3 cm in diameter and have 5 or more mitoses per 10 high-power fields, moderate or marked cytological atypia and infiltrating margins be regarded as leiomyosarcoma {2879}. An epithelioid variant with a myxoid stroma has also been described {456}. As in the uterus, occasional sarcomas arise from leiomyomas {1682}. Differential diagnosis L e i o m y o s a rcomas should be diff e re n t i a t- ed from the benign condition of postoperative spindle cell nodule {2861}. The latter is a localized non-neoplastic lesion composed of closely packed spindle-shaped cells and capillaries occurring several weeks to several months postoperatively in the region of an excision. It may closely resemble a l e i o m y o s a rcoma, but the history of a recent operation at the same site facilitates its diagnosis. Prognosis and predictive factors Leiomyosarcomas are treated primarily by radical surgical excision (vaginectom y, hysterectomy and pelvic lymphadenectomy). In the only large series of 60 smooth muscle tumours, both benign and malignant, only 5 neoplasms recurred, and in one of these, a tumourwith an infiltrative margin, the patient died of lung metastases {2879}. Endometrioid stromal sarcoma, low grade Definition A sarcoma with an infiltrating pattern that in its well differentiated form resembles normal endometrial stromal cells. Histopathology Low grade endometrioid stromal sarcomas have been rarely encountered in the vagina and resemble their counterparts in the endometrium. In two cases the tumours appear to have arisen fro m endometriosis {245}. Before concluding that such a neoplasm is primary in the vagina, an origin within the uterus should be excluded {633,1051,2226}. The term undifferentiated vaginal sarcoma is preferred for the high grade lesions. Undifferentiated vaginal sarcoma Definition A sarcoma with an infiltrating pattern composed of small spindle-shaped cells lacking specific features. Histopathology U n d i ff e rentiated vaginal sarcomas are r a re, polypoid or diffusely infiltrating lesions. Spindle to stellate cells with scanty cytoplasm are arranged in sheetlike, fascicular or storiform patterns. The cells exhibit various degrees of nuclear pleomorphism and hyperchromasia. The mitotic index is ≥10 per 10 high power fields. Mesenchymal tumours 303
Prognosis and predicitive factors Death from re c u r rent or metastatic tumour has occurred within 2 years of treatment in about 50% of patients {20, 3236}. Rare malignant mesenchymal tumours R a re examples of malignant schwannoma {633}, fibro s a rcoma {2160}, malignant fibrous histiocytoma {3078}, a n g i o s a rcoma {1804,2298,2931}, alveolar soft part sarcoma {402}, synovial sarcoma {2095}, malignant peripheral nerve sheath tumour {2226} and unclassifiable s a rcoma {633} have all been described in the vagina, but they do not exhibit unique clinical or morphological feat u re s . Benign mesenchymal neoplasms Of the benign tumours only leiomyomas are relatively common. ICD-O codes Leiomyoma 8890/0 Genital rhabdomyoma 8905/0 Deep angiomyxoma 8841/1 Clinical features Most benign tumours are asymptomatic, but depending on their size and position they may cause pain, bleeding, dysp a reunia and urinary or rectal sympt o m s . Leiomyoma Definition A benign neoplasm composed of smooth muscle cells having a variable amount of fibrous stroma. Aetiology There are virtually no clues to the pathogenesis of this group of tumours. Rare leiomyomas may recur in one or more p regnancies suggesting horm o n e dependency {2501}. Histopathology Vaginal leiomyomas resemble their uterine counterparts. A case of bizarre (symplastic) leiomyoma has been described {264}. Histogenesis The histogenesis of smooth muscle tumours is not clear, but myoepithelial cells such as are found in smooth muscle cells of venules or of the vaginal muscularis and myofibroblasts have all been implicated. Prognosis and predictive factors Nearly all are treated by local excision {1682,2486,2523}. An occasional tumour, especially if large, may recur {685,1682}. Genital rhabdomyoma Definition An uncommon benign tumour of the lower female genital tract showing skeletal muscle differentiation. Epidemiology About 20 cases have been re p o rt e d {1313,2812}. Fig. 6.18 Genital rhabdomyoma. The tumour is composed of individual mature rhabdomyoblasts separated by abundant connective tissue stroma. Clinical features These tumours occur in middle age women (range 30-48 years) and present as a well defined, solitary mass with the clinical appearance of a benign vaginal polyp {1397}. Macroscopy Genital rhabdomyomas are solitary, nodular or polypoid, ranging in size from Epidemiology A p p roximately 300 cases of vaginal leiomyoma have been re p o rt e d . Although the age at presentation ranges from 19-72 years, they typically occur during reproductive life (mean age 44 years) {2879}. Leiomyomas of the vagina are not related to those of the uterus, either in frequency or in racial distribution, the White to Black ratio for uterine and vaginal leiomyomas being 1:3 and 4:1 respectively {222}. Fig. 6.19 Genital rhabdomyoma. The tumour is composed of individual mature rhabdomyoblasts with cross striations in the cytoplasm and bland nuclei without mitotic activity. 304 Tumours of the vagina
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Previous volumes in this series Kle
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World Health Organization Classific
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Published by IARC Press, Internatio
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CHAPTER 1 Tumours of the Breast Can
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TNM classification of carcinomas of
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Invasive breast carcinoma I.O. Elli
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Rapid growth and greater adult heig
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tives, administrative and clerical
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Grade 1 - well differentiated: 3-5
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ent and this is occasionally extens
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Most melanotic tumours of the breas
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A Fig. 1.22 A Classic invasive lobu
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yet others at 90% {97,2147}. For pr
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Fig. 1.27 Medullary carcinoma. The
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myoepithelial cells in fibro a d e
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A Fig. 1.33 Neuroendocrine carcinom
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Macroscopy Fisher et al. reported t
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Fig. 1.39 Apocrine carcinoma. Note
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cells contain intracytoplasmic lume
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A Fig. 1.50 Carcinosarcoma. A Two a
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A B C Fig. 1.75 Lobular neoplasia.
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Intraductal proliferative lesions F
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A B absence of either microcalcific
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Fig. 1.83 Atypical ductal hyperplas
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A B Fig. 1.88 Large excision biopsy
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unusual variants as well. Using thi
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esemble those identified in invasiv
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A B Fig. 1.97 Microinvasive carcino
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A Papilloma may be subject to morph
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A B C Fig. 1.103 Papillary intraduc
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colour measuring from 0.5 to 12 cm.
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Immunoprofile The cases studied by
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Glycogen-rich, clear cell carcinoma
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Differential diagnosis There may be
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quality metaphase spreads from an i
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Fig. 1.67 Lobular carcinoma of brea
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detectable distant metastasis displ
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detected at a late stage as small t
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Extent of ductal carcinoma in situ
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Benign epithelial proliferations G.
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Fig. 1.112 Microglandular adenosis.
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A Apocrine adenoma ICD-O code 8401/
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A B Fig. 1.128 Adenomyoepithelioma,
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Mesenchymal tumours M. Drijkoningen
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1113,1275}. There is a complex patt
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A B Fig. 1.137 A Lipoma. Intraparen
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Fig. 1.141 Angiosarcoma after breas
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A Fig. 1.144 Mammary osteosarcoma.
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Fibroepithelial tumours J.P. Belloc
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aged women (average age of presenta
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lished data suggests a 21% rate of
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A Fig. 1.157 Syringomatous adenoma
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Malignant lymphoma and metastatic t
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used. Inflammatory conditions in th
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male population both in the USA and
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CHAPTER 2 Tumours of the Ovary and
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Polyembryoma 9072/3 Non-gestational
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Surface epithelial-stromal tumours
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A Fig. 2.04 A Serous borderline tum
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A Fig. 2.06 Serous borderline tumou
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A Fig. 2.10 Invasive peritoneal imp
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Fig. 2.15 Mucinous carcinoma with i
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Fig. 2.20 Mucinous endocervical-lik
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A Fig. 2.28 Mucinous cystic tumour
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early secretory endometrium {2605}.
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IV, 6% {2233}. Patients with grade
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A Fig. 2.37 A This polypoid intracy
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Fig. 2.40 Endometrioid cyst with at
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1 tumours are extremely rare. Almos
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Fig. 2.50 Borderline Brenner tumour
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express thrombomodulin and have bee
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serous and transitional cell carcin
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is yellow to tan with a variable ad
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Genetic susceptibility JGCTs may pr
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Clinical features F i b romas may b
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distinguishes this tumour from the
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A Fig. 2.77 A Retiform Sertoli-Leyd
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Mean ages of 21 and 38 years and me
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Tumours unassociated with PJS form
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mal origin without crystals of Rein
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Germ cell tumours F. Nogales A. Tal
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cases, anisokaryosis has no prognos
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ation may coexist in the same neopl
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Table 2.06 Grading of ovarian immat
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A B Fig. 2.102 A Mature cystic tera
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Diagnostic procedures Elevated urin
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associated with mature teratomas sh
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atives intimately admixed. The germ
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Fig. 2.113 Mixed germ cell-sex cord
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A Fig. 2.116 A Adenomatous hyperpla
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Fig. 2.117 Small cell carcinoma, hy
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Clinical features Adenoid cystic-li
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Histopathology This epithelial tumo
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sis. Corpus luteum of pregnancy has
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Lymphomas and leukaemias L.M. Roth
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Secondary tumours of the ovary J. P
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Occasionally, the colonic adenocarc
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Peritoneal tumours S.C. Mok J.O. Sc
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A B Fig. 2.140 Cystic adenomatoid m
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whelmingly poor {1038,1547,2310}. H
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CHAPTER 3 Tumours of the Fallopian
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TNM and FIGO classification of carc
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Endometrioid adenocarcinoma Endomet
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Two examples of adenofibroma of bor
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A Fig. 3.11 Adenomatoid tumour. A T
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Clinical features Patients range in
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Fig. 3.16 Uterus-like mass. The cys
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CHAPTER 4 Tumours of the Uterine Co
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TNM and FIGO classification of non-
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Epithelial tumours and related lesi
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endometrioid adenocarcinoma fro m a
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fers from the prototypical type I e
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the ovary and bladder. Unlike prima
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schema {1535,2602}. Although this c
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Table 4.03 Altered gene function in
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Mesenchymal tumours and related les
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areas are limited to less than 30%
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A B C Fig. 4.30 Leiomyosarcoma. A T
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e used sparingly and is reserved fo
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A B Fig. 4.38 Leiomyoma with perino
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cytological atypia, tumour cell nec
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Mixed epithelial and mesenchymal tu
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Prognosis and predictive factors Th
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tumours may superficially invade th
Page 252 and 253: A Fig. 4.46 A Gestational choriocar
Page 254 and 255: A Fig. 4.49 A Classic complete hyda
Page 256 and 257: Sex cord-like, neuroectodermal and
Page 258 and 259: Secondary tumours of the uterine co
Page 260 and 261: WHO histological classification of
Page 262 and 263: Epithelial tumours M. Wells J.M. Ne
Page 264 and 265: Fig. 5.04 Mechanisms of human papil
Page 266 and 267: Fig. 5.08 Keratinizing squamous cel
Page 268 and 269: in their Asian population {2957}. I
Page 270 and 271: have a strong association with high
Page 272 and 273: e red by squamous epithelium {380}.
Page 274 and 275: endometrioid adenocarcinoma of the
Page 276 and 277: metrial epithelium. In some cases t
Page 278 and 279: with distinct cell borders and a gr
Page 280 and 281: Mesenchymal tumours M.L. Carcangiu
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Page 284 and 285: Mixed epithelial and mesenchymal tu
Page 286 and 287: Fig. 5.44 Wilms tumour. The tumour
Page 288 and 289: A Fig. 5.47 Implant of endometrial
Page 290 and 291: CHAPTER 6 Tumours of the Vagina Alt
Page 292 and 293: Epithelial tumours E.S. Andersen A.
Page 294 and 295: Aetiology The fact that both VAIN a
Page 296 and 297: Fig. 6.10 Fibroepithelial polyp.A m
Page 298 and 299: er of mitoses varies but is usually
Page 300 and 301: ICD-O codes Adenosquamous carcinoma
Page 304 and 305: 1-11 cm. They may arise anywhere in
Page 306 and 307: elsewhere in the female genital tra
Page 308 and 309: A Fig. 6.24 Yolk sac tumour. A The
Page 310 and 311: g rowing in sheets. Some may have s
Page 312 and 313: WHO histological classification of
Page 314 and 315: Epithelial tumours E.J. Wilkinson M
Page 316 and 317: even with additional sectioning, it
Page 318 and 319: type) is a highly diff e rentiated
Page 320 and 321: Clinical features Bartholin gland c
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Page 324 and 325: Mesenchymal tumours R.L. Kempson M.
Page 326 and 327: Table 7.03 Differential diagnosis o
Page 328 and 329: Prognosis and predictive factors A
Page 330 and 331: Table 7.04 Clark levels of cutaneou
Page 332 and 333: A Fig. 7.23 Vulvar peripheral primi
Page 334 and 335: Familial aggregation of cancers of
Page 336 and 337: BRCA1 syndrome Definition Inherited
Page 338 and 339: dispose individuals to the developm
Page 340 and 341: Fig. 8.05 To assess whether wild-ty
Page 342 and 343: Fig. 8.07 Factors that modify risk
Page 344 and 345: BRCA2 syndrome R. Eeles S. Piver S.
Page 346 and 347: tubal or ovarian carcinomas. Howeve
Page 348 and 349: in typical nuclear foci that may re
Page 350 and 351: Breast tumours Frequency Breast can
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Fig. 8.14 Codon distribution of som
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Breast tumours Age distribution and
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Hereditary non-polyposis colon canc
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essary in the evaluation of the pat
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Age distribution and penetrance Mos
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Contributors Dr Vera M. ABELER** De
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Dr Carlo LA VECCHIA Laboratory of E
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Dr Manuel TEIXEIRA Department of Ge
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02.100 Dr. A. Ostor 02.101 Dr. F.A.
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52. Akhtar M, Robinson C, Ashraf Al
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180. Bapat K, Brustein S (1989). Ut
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307. Bolis GB, Maccio T (2000). Cle
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436. Chang J, Sharpe JC, A'Hern RP,
Page 378 and 379:
562. Costa MJ, Ames PF, Walls J, Ro
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689. Di Domenico A, Stangl F, Benni
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820. Falck J, Petrini JH, Williams
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943. Gad A, Azzopardi JG (1975). Lo
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1067. Grimes MM (1992). Cystosarcom
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1197. Herod JJ, Shafi MI, Rollason
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1323. Jacques SM, Qureshi F, Ramire
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1449. Khalifa MA, Mannel RS, Harawa
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1564. Lagios MD (1977). Multicentri
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1687. Loman N, Johannsson O, Kristo
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1807. McCluggage G, McBride H, Maxw
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1937. Mukai M, Torikata C, Iri H (1
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2056. Norris HJ, Taylor HB (1967).
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2180. Park JS, Jones RW, McLean MR,
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2304. Puls LE, Hamous J, Morrow MS,
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2 4 3 4 . Rosen PP, Groshen S, Saig
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2559. Schlesinger C, Silverberg SG
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2686. Silverberg SG (1999). Protoco
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2806. Stutz JA, Evans AJ, Pinder S,
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2942. Tornos C, Silva EG, Ordonez N
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3061. Wargotz ES, Norris HJ (1990).
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3189. Yoshioka T, Tanaka T (2000).
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References 425
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Biphasic teratomas, 168 BLM, 341, 3
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G GADD45, 343, 353 GCDFP-15, 25, 33
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MPNST, see Malignant peripheral ner
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Small cell / oat cell carcinoma, 32
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