Vulvar “proximal-type” epithelioid sarcoma: report of a case and review of the literature

Abstract

Abstract Background The “proximal-type” epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior. Case We report of a case of a 63 years old woman with a vulvar “proximal-type” epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease. Conclusion Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy. Virtual slides The virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/1508554852942125