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[Sclerosing epithelioid fibrosarcoma].

L Boudová¹, M Michal, Z Kinkor

¹Siklův patologicko-anatomický ústav, LF UK a Fakultní nemocnice, Plzen. boudova@fnplzen.cz

Ceskoslovenska Patologie

|January 30, 2002

Summary

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumor. This study details 6 new cases, highlighting their histological features, immunohistochemistry, and clinical outcomes, including recurrence and metastasis.

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Area of Science:

Oncology
Pathology
Soft Tissue Tumors

Context:

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue neoplasm.
Only 39 cases have been reported since its initial description in 1995.
This study presents 6 additional cases to expand the understanding of SEF.

Purpose:

To describe the clinicopathological features of 6 new cases of SEF.
To analyze immunohistochemical findings and proliferative activity (Ki-67).
To report on patient follow-up, including recurrence and metastasis patterns.

Summary:

Six SEF cases (3 male, 3 female, ages 22-79) occurred in soft tissues of extremities and trunk.
Histology revealed epithelioid cells in a dense hyalinized stroma, with low mitotic activity.

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Immunohistochemistry showed vimentin positivity, with some cases positive for EMA and cytokeratins. Proliferation index (Ki-67) was low (1-6%).

Follow-up in 5 patients showed local recurrences and distant metastases in 2 cases, with one fatality.

Impact:

Adds to the limited literature on sclerosing epithelioid fibrosarcoma.
Provides detailed clinicopathological and immunohistochemical data for this rare tumor.
Highlights the potential for recurrence and metastasis, informing differential diagnosis and patient management.