Vulvar “proximal-type” epithelioid sarcoma: report of a case and review of the literature
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
Diagnostic Pathology 2013, 8:122 doi:10.1186/1746-1596-8-122Published: 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.
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