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

Lodovico Patrizi2, Giacomo Corrado1*, Maria Saltari2, Letizia Perracchio3, Chiara Scelzo2, Emilio Piccione2 and Enrico Vizza4

Author Affiliations

1 Department of Oncological Surgery, Gynecologic Oncology Unit, “Regina Elena” National Cancer Institute, Via Elio Chianesi 53, Rome, 00144, Italy

2 Surgery Department, Gynecology Section and Obstetrics, Tor Vergata University, Rome, Italy

3 Pathology Department, “Regina Elena” National Cancer Institute, Rome, Italy

4 Surgery Department, Gynecologic Oncology Unit, “Regina Elena” National Cancer Institute, Rome, Italy

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Diagnostic Pathology 2013, 8:122  doi:10.1186/1746-1596-8-122

Published: 25 July 2013



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.


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.


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: webcite

Vulvar “proximal-type” epithelioid sarcoma; Radical vulvectomy; Vulvar cancer