Sclerosing epithelioid fibrosarcoma – a rare variant with myriads of differentials

Abstract

Sclerosing Epithelioid Fibrosarcoma is a rare form of soft tissue sarcoma, with increased propensity for local recurrence and metastases mainly pulmonary and pleural in about 50% of cases. It carries morphological resemblance with many sarcomas, hence detailed and comprehensive immunohistochemical examination is mandatory for definitive diagnosis. Hereby, we present a case in a 39 year old male, who presented with a painless right thigh swelling. The histopathological examination revealed nests of epithelioid cells embedded in a densely sclerotic matrix, and immunohistochemistry showed MUC4 and Vimentin positivity, rendering the final diagnosis of Sclerosing Epithelioid Fibrosarcoma.