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WHO Classification of Tumours
Sclerosing epithelioid fibrosarcoma
Connective, subcutaneous and other soft tissues


Definition

Sclerosing epithelioid fibrosarcoma (SEF) is a rare, invariably deep-seated fibrosarcoma variant, arising mainly in the lower extremities and limb girdles, followed by trunk, upper extremities, and the head and neck area. The lesions are generally well circumscribed, lobulated or multinodular and range in size from 2 to 22 cm (median 7-10 cm). SEF is histologically characterized by small epithelioid tumour cells arranged in nests and cords that are embedded within a densely sclerotic collagenous matrix. Myxoid, cystic and calcified areas are common
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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SEF occurs over a wide age spectrum, the median age is 45 years. Local recurrence occurs in >50% of cases, and >40% of tumours metastasize, usually to lungs, pleura and bone. After 11 years, 50% of patients have either died or have persistent or recurrent tumour

Click to access Pubmed
Meis-Kindblom JM, Kindblom LG, Enzinger FM (1995)
Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma.
Am J Surg Pathol 19: 979-93



. Adverse prognostic factors include large tumour size, proximal tumour site, male sex, local recurrences, and metastases
Click for details
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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