Pathologica 4-07.pdf - Pacini Editore

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Cranio-axial chordomas. A 15-year clinicopathological
experience at the “Casa Sollievo
della Sofferenza” hospital
M. Bisceglia * , C. Clemente * , M. Vairo * , L. Di Candia * , G.
Giannatempo ** , M. Bianco *** , V.A. D’Angelo *** G. Pa-
* ****
squinelli
Departments of * Pathology, ** Radiology, and *** Neurosurgery,
IRCCS “Casa Sollievo della Sofferenza” Hospital, San
Giovanni Rotondo, Italy; **** Department of Clinical Pathology,
Policlinico “S. Orsola” Hospital, Bologna, Italy
Introduction. Chordoma (CD) is a rare, slowly growing, malignant
tumor with phenotypic epithelial features arising
from notochordal rests, and occurring in several anatomic locations.
Axial skeleton involvement from the clivus to the tip
of coccyx is the standard, but even heterotopic CD are on
record (paraxial-lateral bone and soft tissue occurrence). CD
represents only 1% to 4% of all primary bone tumors 1 . The
yearly estimated incidence is < 1 case per million individuals,
and most large general hospitals see 1 case every 1-2
years. CD is a tumor of adults and the elderly (peak incidence:
VI decade) and very rare under 20 years. Both sexes
are affected. The clinical presentation is diverse and related
to the tumor location. Histological variants have been described:
i.e., atypical, anaplastic, spindle cell, and dedifferentiated
(DD) – with 53 cases on record of the latter as either
primary or secondary to radiation 2 .
Design. The clinicopathologic records of 24 total CDs seen
over the last 15 years, focusing on their histologies, were reviewed.
All cases underwent imaging studies. The age ranged
from 9 years to 80 years (mean 47.5 years); 4 of them occurred
under 30 years of age (1 case in the I and 3 in the III
decade). Male to female ratio was 11:13. All patients complained
of diverse site-based symptomatology. The tumor location
was cranial in 13 cases, vertebral in 6, and sacral in 4;
1 case was heterotopic (maxillary bone). All cases underwent
surgery or needle biopsy. 6 cases had multiple biopsy (preoperative;
recurrence; regional metastasis). Tumor size
ranged from 2 (maxillary bone) to 15 cm (sacral). All cases
were histologically diagnosed and immunoistochemically assessed.
3 cases were examined by electron microscopy (EM).
1 case was left with uncertain diagnosis (sacral CD vs. h.g.
myxoid chondrosarcoma) and excluded from this review.
Results. At histology a classic pattern was seen in 19 cases.
Atypical or anaplastic features were seen in 4 cases. 1 case
with anaplastic features was mainly a primary DD-CD of the
POSTERS
T9 vertebra. Immunohistochemistry (vimentin, EMA, S-100
pr, CK w.s. + ty) was of support in all cases and decisive in
some. EM was of invaluable help in 1 intracranial chondroid
CD, and in 1 L5 vertebral CD with marked epithelial features
by showing the classical RER-mitochondrial complexes and
other suggestive features; focal rhabdomyosarcomatous differentiation
in the DD-CD was also first recognized on EM.
The differential diagnosis often included metastatic mucinous
carcinoma; metastatic renal cell carcinoma was considered
in the L5 vertebral case; pleomorphic sarcoma was first
suspected in the DD-CD.
Conclusions. CD should always be suspected in any case of
epithelial-looking tumors with extracellular myxoid matrix
or mucocellular features involving the cranioaxial skeleton.
Metastatic carcinoma both mucinous and non-mucinous may
be mimicked by CD. Chondroid CD and DD-CD might be
undistinguishable from myxoid chondrosarcoma and other
sarcomas.
References
1 Dorfman HD, Czerniak B. Bone Tumors. St Louis, MO: Mosby 1998,
p. 1139-52.
2 Bisceglia M, et al. Ann Diagn Pathol 2007, in press.
Metastasis of high grade renal cell
carcinoma, clear cell type, in fibrous
dysplasia with superimposed giant cell
reparative granuloma
C. Rizzardi, M. Schneider, M. Melato
DIA di Anatomia Patologica e Medicina Legale, Università
di Trieste, Trieste, Italia
A case of monostotic fibrous dysplasia in a 54-year-old man
complaining of severe pain in the right hip is presented.
Imaging findings demonstrated an extremely aggressive lesion
involving bones, liver, lungs, and lymph nodes, and suggested
the possibility of sarcomatous transformation. Histological
examination established a diagnosis of metastatic
high grade renal cell carcinoma, clear cell type, and demonstrated
the presence of superimposed giant cell reparative
granuloma. It is a rare example of giant cell reparative granuloma
arising in a long bone and in association with fibrous
dysplasia. The clinical, radiographic, and histopathologic
features of fibrous dysplasia and giant cell reparative granuloma
are reviewed.