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Vulvar epithelioid sarcoma: A case report with literature review
Joseph J Noh1, Jung-Eun Jeon1, Hera Jung2
1Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Objective:
To report a case of vulvar epithelioid sarcoma, a rare type of tumor that has been reported in 37 cases in the English literature to date.
Case Report:
We report three cases of vulvar epithelioid sarcoma, proximal type. Wide excisions of the mass were performed with margins free of tumor in all three cases.
Conclusion:
Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. Locoregional lymph node involvement, vascular invasion, tumor size larger than 2 cm, deep localization, presence of necrosis, and a high mitotic index are known as poor prognostic factors. Adjuvant radiotherapy is advisable in the presence of a high-grade tumor or positive margins. The beneficial effect of adjuvant chemotherapy is not well established. Treatment decisions should be made based on the individual case presentation and pathology evaluation.
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