Key result
Wide excision of the mass resulted in tumor-free margins in all three reported cases of proximal type vulvar epithelioid sarcoma.
Case Report (n=3)
Wide excision with tumor-free margins is a feasible treatment for vulvar epithelioid sarcoma, though adjuvant therapy decisions remain individualized due to lack of evidence.
Supports wide excision for local control in rare vulvar sarcoma; leaves open adjuvant strategies and long-term outcomes.
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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Noh et al. (2021) conducted a case report in Vulvar epithelioid sarcoma (n=3). Wide excision was evaluated. Wide excision of the mass resulted in tumor-free margins in all three reported cases of proximal type vulvar epithelioid sarcoma.
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