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Case Reports in Obstetrics and Gynecology
Volume 2015, Article ID 971217, 5 pages
Case Report

Extremely Rare Case of Vulvar Myxoid Epithelioid Sarcoma

1Gynecologic Department, Bissaya Barreto Maternity Hospital, CHUC, 3000-059 Coimbra, Portugal
2Gynecologic Oncologic Department, Barretos Cancer Hospital, SP, Brazil
3Radiology Department, Barretos Cancer Hospital, SP, Brazil
4Pathology Department, Barretos Hospital, SP, Brazil

Received 24 November 2014; Revised 27 January 2015; Accepted 27 January 2015

Academic Editor: Yoshio Yoshida

Copyright © 2015 Joana Lima Rego et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Epithelioid sarcoma is a distinct sarcoma type with specific morphology and immunophenotype. An epithelioid sarcoma of the vulva is an extremely rare and aggressive tumor and most commonly occurs on the labia majora in women of reproductive age. Only few cases have been reported, especially with the presence of focal myxoid changes. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Optimal treatment is not well established due to its rarity. We report a successfully approached case of vulvar epithelioid sarcoma that occurred in a 34-year-old female patient, treated with wide local excision, and review of the current medical literature.