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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

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