Case report of epithelioid sarcoma of the vulva

Epithelioid sarcoma is an extremely rare malignant mesenchymal tumor, accounting for less than 1% of all types of sarcomas. When it occurs in the vulva, it is even less common, exhibiting high invasiveness and a significant risk of recurrence. Epithelioid sarcoma that develops in the deep layers of the vulva is often insidious and may clinically present as a vulvar abscess, which can easily lead to misdiagnosis as a benign lesion.

A 56-year-old female patient presented with a rapidly growing, painful mass measuring 3 cm in diameter on the left vulva. She visited multiple hospitals over the course of 3 months and received anti-inflammatory treatment as well as incision and drainage for a local abscess, but showed no improvement. The patient eventually sought care at our hospital due to progressive ulceration of the vulva, increasing size of the lesion, and swelling of the buttock skin accompanied by septic shock. After stabilizing her general condition, an excision of the vulvar lesion and a fasciotomy for decompression and drainage of the buttock were performed. Postoperative pathology confirmed a diagnosis of vulvar epithelioid sarcoma (proximal type). The patient subsequently died from septic shock associated with multiple organ failure.

From a clinical perspective, vulvar epithelioid sarcoma can present similarly to various benign vulvar lesions, such as Bartholin gland abscesses and infectious granulomas, leading to potential misdiagnosis. Here, we share our experience in the diagnosis and management of a patient with vulvar epithelioid sarcoma to provide a cautionary perspective for the clinical care of comparable cases.