title: Proximal-type epithelioid sarcoma -- case report.
abstract: Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.
authors.affiliation: Tropical Medicine Foundation of the Amazon State, Manaus, AM, Brazil. m.n.souza.santos@gmail.com
authors.forename: Luciana Mendes dos
authors.initials: LM
authors.lastname: Santos
authors.forename: Lisiane
authors.initials: L
authors.lastname: Nogueira
authors.forename: Christiane Yuri
authors.initials: CY
authors.lastname: Matsuo
authors.forename: Carolina
authors.initials: C
authors.lastname: Talhari
authors.forename: Mônica
authors.initials: M
authors.lastname: Santos
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000556:secondary; D002081:Buttocks; D017809:Fatal Outcome; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D035583:Rare Diseases / Q000473:pathology*; D012509:Sarcoma / Q000473:pathology* / Q000556:secondary; D012878:Skin Neoplasms / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000473:pathology*