Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
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A<br />
Fig. 2.15 A Infiltrating urothelial carcinoma of the bladder, plasmocytoid variant. B Plasmacytoid variant of<br />
urothelial carcinoma of the urinary bladder.<br />
exclusive component in a small biopsy<br />
sample. The importance of recognizing<br />
these variants lies in not mistaking them<br />
as a lymphoma or plasmacytoma.<br />
Limited information is available about the<br />
outcome of patients with these variants of<br />
urothelial carcinoma. Of 6 cases reported<br />
by Tamboli et al. {2571} 4 died of their<br />
disease, one died post-operatively and<br />
one is alive without evidence of disease.<br />
Sarcomatoid variant<br />
(with/without heterologous elements)<br />
The term sarcomatoid variant of urothelial<br />
carcinoma should be used for all<br />
biphasic malignant neoplasms exhibiting<br />
morphologic and/or immunohistochemical<br />
evidence of epithelial and mesenchymal<br />
differentiation (with the presence or<br />
absence of heterologous elements<br />
acknowledged in the diagnosis). There is<br />
considerable confusion and disagreement<br />
in the literature regarding nomenclature<br />
and histogenesis of these<br />
tumours. In some series, both carcinosarcoma<br />
and sarcomatoid carcinoma<br />
are included as "sarcomatoid carcinoma"<br />
{2175}. In others they are regarded as<br />
separate entities.<br />
The mean age is 66 years (range, 50-77<br />
years old) and most patients present with<br />
hematuria {1555,2175}. A previous history<br />
of carcinoma treated by radiation or<br />
the exposition to cyclophosphamide<br />
therapy is common {1551}. Rare examples<br />
of carcinosarcoma and sarcomatoid<br />
carcinomas have been described in the<br />
ureter and the renal pelvis {1549}.<br />
The gross appearance is characteristically<br />
"sarcoma-like", dull grey with infiltrative<br />
margins. The tumours are often polypoid<br />
with large intraluminal masses.<br />
Microscopically, sarcomatoid carcinoma<br />
is composed of urothelial, glandular or<br />
small cell component showing variable<br />
degrees of differentiation {1555}. A small<br />
B<br />
A<br />
C<br />
subset of sarcomatoid carcinoma may<br />
have a prominent myxoid stroma {1238}.<br />
The mesenchymal component most frequently<br />
observed is an undifferentiated<br />
high grade spindle cell neoplasm. The<br />
most common heterologous element is<br />
osteosarcoma followed by chondrosarcoma,<br />
rhabdomyosarcoma, leiomyosarcoma,<br />
liposarcoma angiosarcoma or<br />
multiple types of heterologous differentiation<br />
may be present {957,1238,1549,<br />
1555,2175}. By immunohistochemistry,<br />
epithelial elements react with cytokeratins,<br />
whereas stromal elements react<br />
with vimentin or specific markers corresponding<br />
to the mesenchymal differentiation.<br />
The sarcomatoid phenotype<br />
retains the epithelial nature of the cells by<br />
immunohistochemistry or electronmicroscopy<br />
{1549,1555}. Recent molecular<br />
studies, strongly argue for a monoclonal<br />
origin of both components {957}.<br />
The cytological atypia of sarcomatoid<br />
carcinoma excludes non-neoplastic<br />
lesions such as the postoperative spindle<br />
cell nodule and inflammatory<br />
pseudotumour {1161,1550}. Sarcomatoid<br />
carcinoma should be distinguished<br />
from the rare carcinoma with metaplastic,<br />
benign-appearing bone or cartilage in<br />
the stroma or those showing other pseudosarcomatous<br />
stromal reactions.<br />
Nodal and distant organ metastases at<br />
diagnosis are common {957,1555,1960,<br />
2175} and 70% of patients died of cancer<br />
at 1 to 48 months (mean 17 months) {1555}<br />
Urothelial carcinoma with giant cells<br />
High grade urothelial carcinoma may<br />
contain epithelial tumour giant cells or the<br />
tumour may appear undifferentiated<br />
resembling giant cell carcinoma of the<br />
lung. This variant is very infrequent. It<br />
must be distinguished from occasional<br />
cases showing giant cells (osteoclastic or<br />
foreign body type) in the stroma or urothelial<br />
carcinoma showing trophoblastic differentiation.<br />
In some cases the giant cell<br />
reaction is so extensive that it may mimic<br />
giant cell tumour of the bone {2948}.<br />
Fig. 2.16 A Infiltrative urothelial carcinoma. Sarcomatoid variant without heterologous elements showing<br />
spindle cell morphology. B Infiltrating urothelial carcinoma of the bladder. Sarcomatoid variant with heterologous<br />
smooth muscle elements. C Immunohistochemical expression of cytokeratin AE1/AE3 in a case<br />
of sarcomatoid carcinoma of the urinary bladder (same case as in panel A). D Immunohistochemical<br />
expression of smooth muscle actin of the sarcomatoid carcinoma shown in panel B.<br />
B<br />
D<br />
102 Tumours of the urinary system