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    A primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation.

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    Authors
    Wang, Guofeng
    Eyden, Brian P
    Affiliation
    Department of Pathology, Second Affiliated Hospital of the Medical College, Zhejiang University, Hangzhou, China. wgf2202@yahoo.com.cn
    Issue Date
    2010-04
    
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    Abstract
    Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma, described initially by Meis-Kindblom et al. in 1995 (Meis-Kindblom JM, Kindblom L-G, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979-993): more than 80 cases have been documented clinicopathologically since. Bone is a rare primary site for SEF, with only 2 cases so far reported. This paper documents the detailed clinical, histological, immunohistochemical, and ultrastructural features of a case occurring in the pubic bone of a 57-year-old diabetic woman presenting with a history of pain and compromised mobility involving her hip. Radiology revealed a destructive lesion in the right pubic bone. The lesion was resected, and 7 months postoperatively it recurred. The patient died following metastases to multiple bony sites and liver, some 4 years after the onset of symptoms. Histologically, the tumor was consistent with SEF, although it showed some anomalous immunostaining, which, however, is typical of the tumor (for example, for S-100 protein and epithelial membrane antigen). By electron microscopy, some rough endoplasmic reticulum was present, but also tonofibrils and desmosomes. The overall features were of an SEF with the ultrastructural but incomplete immunohistochemical evidence for divergent epithelial differentiation. The differential diagnosis of this tumor is discussed.
    Citation
    A primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation. 2010, 34 (2):99-104 Ultrastruct Pathol
    Journal
    Ultrastructural Pathology
    URI
    http://hdl.handle.net/10541/109362
    DOI
    10.3109/01913121003605576
    PubMed ID
    20192707
    Type
    Article
    Language
    en
    ISSN
    1521-0758
    ae974a485f413a2113503eed53cd6c53
    10.3109/01913121003605576
    Scopus Count
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