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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Malignant fibrous histiocytoma<br />
J. Cheville<br />
Definition<br />
Malignant fibrous histiocytoma (MFH) is<br />
a malignant mesenchymal neoplasm<br />
occurring in the urinary bladder composed<br />
of fibroblasts and pleomorphic<br />
cells with a prominent storiform pattern.<br />
ICD-O code 8830/3<br />
Synonym<br />
Undifferentiated high grade pleomorphic<br />
sarcoma.<br />
Epidemiology<br />
Malignant fibrous histiocytoma is one of<br />
the most frequent soft tissue sarcomas,<br />
and in some series, the second most frequent<br />
sarcoma of the urinary tract in<br />
adults {1410}. It is difficult to determine<br />
the incidence of urinary bladder malignant<br />
fibrous histiocytoma as it is likely<br />
that several tumours previously reported<br />
as malignant fibrous histiocytoma are<br />
sarcomatoid urothelial carcinoma.<br />
Malignant fibrous histiocytoma more frequently<br />
affects men, and is most common<br />
in patients in their 5th to 8th decade.<br />
Clinical features<br />
Patients present with haematuria.<br />
Macroscopy<br />
Similar to other sarcomas of the urinary<br />
bladder, most malignant fibrous histiocytomas<br />
are large but tumours as small as<br />
1 cm have been reported.<br />
Histopathology<br />
All subtypes of malignant fibrous histiocytoma<br />
have been described involving<br />
the bladder including myxoid, inflammatory,<br />
storiform-fascicular, and pleomorphic<br />
{809,1410,1935}. Malignant fibrous<br />
histiocytoma must be separated from<br />
sarcomatoid urothelial carcinoma as well<br />
as reactive spindle cell proliferations of<br />
the bladder. The much more commonly<br />
encountered sarcomatoid urothelial carcinoma<br />
can be associated with a malignant<br />
epithelial component, and stains<br />
positively for the immunohistochemical<br />
markers of epithelial differentiation such<br />
as cytokeratin {1038,1555,2038}. In contrast,<br />
malignant fibrous histiocytoma is<br />
negative for cytokeratin, and can stain for<br />
alpha-1-antichymotrypsin, and CD68.<br />
Reactive spindle cell proliferations lack<br />
the cytologic atypia of malignant fibrous<br />
histiocytoma.<br />
A<br />
Prognosis<br />
The rarity of malignant fibrous histiocytoma<br />
makes it difficult to assess the biologic<br />
behaviour of these tumours. However,<br />
from the limited reports, malignant<br />
fibrous histiocytoma of the bladder<br />
appears aggressive with high local recurrence<br />
rates and metastases similar to<br />
malignant fibrous histiocytoma at other<br />
sites {809}. Treatment consists of resection,<br />
systemic chemotherapy and external<br />
beam radiation. The only patient with myxoid<br />
malignant fibrous histiocytoma of the<br />
bladder has been free to tumour following<br />
surgical resection, local radiation and systemic<br />
chemotherapy for 3 years {809}.<br />
B<br />
C<br />
Fig. 2.79 Malignant fibrous histiocytoma. A Pleomorphic type, showing its characteristic storiform growth<br />
pattern and histologically normal urothelium (right bottom). B Pleomorphic giant cells are a common finding<br />
in this high grade, pleomorphic type, malignant fibrous histiocytoma. C Some pleomorphic cells proliferating<br />
in this malignant fibrous histiocytoma were immunorreactive with Anti-Alpha-1-Antitrypsin antibody.<br />
D Virtually all proliferating cells in this case of malignant fibrous histiocytoma displayed immunorreactivity<br />
with anti-vimentin antibody.<br />
D<br />
Osteosarcoma / Malignant fibrous histiocytoma 143