Dermatofibrosarcoma Protuberans of the Vulva With Myoid Differentiation. (September 2015)

• Record Type: Journal Article
• Authors: Bernárdez, Claudia; Machan, Salma; Molina-Ruiz, Ana María; Pérez de la Fuente, Teresa; Pavón, María; Carrillo, Irene; Fortes, José; Requena, Luis
• Is Part Of: American journal of dermatopathology. Volume 37:Number 9(2015)
• Publication Date: 2015-09
• Subjects: dermatofibrosarcoma protuberans -- giant -- vulva -- fibrosarcoma -- myoid differentiation; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000000289
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract : Abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft-tissue tumor characterized by a relatively high risk for local recurrence and low risk for metastasis. Many histopathologic variants of DFSP have been described, including the fibrosarcomatous and myoid variants, which may obscure the diagnosis in some cases, especially when arising in unusual locations. Of all the variants described so far, the only one with prognostic relevance is the FS-DFSP variant, which implies tumor progression and a higher possibility for metastasis. The authors report a case of a giant DFSP, located on the vulvar area, which histopathologically showed areas of fibrosarcomatous and myoid differentiation, and discuss the importance of the myoid variant in regards of the debated histogenesis of DFSP.