title: ["Proximal type" epithelioid sarcoma of the vulva: differential diagnosis with other extrarenal rhabdoid tumors].
abstract: Proximal type epithelioid sarcoma is a rare neoplasia in which morphological findings are characterized by nodular proliferation of epithelioid cells with focal rhabdoid features. It shares some histological features with other neoplasias and this gives an account of several differential diagnosis with other extrarenal rhabdoid tumors. Immunohistochemical and ultrastructural analysis are important in defining this entity: vimentin, cytokeratin, EMA and often CD34 expression of tumoral cells, moreover ultrastructurally evidence of large paranuclear whorls of intermediate filaments, are requested for diagnosis. A correct diagnostic framing is necessary because of the aggressive clinical behaviour of this tumor, that has a tendency to early spreading. We describe a case of vulvar proximal type epithelioid sarcoma in a 34 years old woman.
authors.affiliation: Istituto di Anatomia Patologica, Dipartimento di Morfologia Umana, Università dell'Insubria, Varese. lele_dainese@hotmail.com
authors.forename: E
authors.initials: E
authors.lastname: Dainese
authors.forename: F
authors.initials: F
authors.lastname: Sessa
authors.forename: C
authors.initials: C
authors.lastname: Riva
authors.forename: C
authors.initials: C
authors.lastname: Placidi
authors.forename: C
authors.initials: C
authors.lastname: Capella
mesh_terms: D000328:Adult; D018952:Antigens, CD34 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D004814:Epidermal Cyst / Q000175:diagnosis; D005260:Female; D006801:Humans; D007382:Intermediate Filaments / Q000473:pathology; D008197:Lymph Node Excision; D018396:Mucin-1 / Q000032:analysis; D009363:Neoplasm Proteins / Q000032:analysis; D012086:Reoperation; D018335:Rhabdoid Tumor / Q000145:classification / Q000175:diagnosis; D012509:Sarcoma / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014846:Vulvar Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery
chemical_list: D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D018396:Mucin-1; D009363:Neoplasm Proteins