Perivascular epithelioid cell tumor of vulva: a rare case

Background: Perivascular epithelioid cell tumor (PEComa) is very rare mesenchymal tumor with an unpredictable natural history. To the best of the present authors’ knowledge, there is only one reported case of PEComa of vulva in the English language medical literature. Case Report: A 49-year-old woman presented with PEComa of vulva. The diagnosis was established postoperatively by immunohistochemistry. To date, two months after operation, the patient is alive with no evidence of recurrence or metastasis. Conclusion: A variety of imaging methods and pathological methods including a surgical resection with clear margin may be necessary in cases of patients with mesenchymal tumor of vulva suspecting PEComa. Urgent diagnosis and treatment of these patients seemed to be critical.

Perivascular epithelioid cell tumor; PEComa; Vulva

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