Sclerosing epitelioid fibrosarcoma: case report and review of the literature

Abstract

The sclerosing epitelioid fibrossarcoma (SEF) is a malignant neoplasm made by nests and cords of small round cells, with clear cytoplasm, exuberant hyalinization and a high degree of necrosis. It seldom occurs in the limbs, trunk and neck. The SEF is a low degree tumor, and is reative to p53. Local recurrence is related to close resection margins. Differencial diagnosis includes nodular fascitis, desmoids tumor, fibrous hystiocitoma, synovial fibrossarcoma, clear cell sarcoma and alveolar rabdomiossarcoma. Tumor size, trunk localization and gender (male) compromises diagnosis. The autors relat a 44 years old patient case, with tumor in the middle anterior portion of the right forearm, and biopsy confirming SEF. Histology, clinical presentation and management options are discussed.