Proximal-type epithelioid sarcoma: A new case report

Abstract

Epithelioid sarcoma (ES) is a rare high-grade soft tissue sarcoma, first described by Enzinger in 1970, classically presenting as a subcutaneous or deep dermal mass in the distal extremities of adolescents and young adults. In 1997, Guillou et al. described the proximal-type epithelioid sarcoma, which is found mostly in the pelvic, perineal regions, and genital tracts of young to middle-aged adults. Epithelioid sarcoma is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. We present, in this paper, a case of proximal-type epithelioid sarcoma of abdominal wall, with an aggressive clinical course, including distant metastasis and death one month after diagnosis. In this particular case, the location is infrequent, and it had not previously been described.