Epithelioid sarcoma of the Bartholin's gland primarily diagnosed as vulvar carcinoma

A case of vulvar epithelioid sarcoma (VES) in a 49-year-old female is described. The disease originated from Bartholin's gland and was originally diagnosed as vulvar squamous cell carcinoma. The patient was treated by radical vulvectomy and bilateral inguinal lymphadenectomy. Multidrug chemotherapy using doxorubicin, cyclophosphamide, vincristine, and dacarbazine was started, but had to be abandoned because of cardiac toxicity. Despite intensive treatment, the patient developed pulmonary metastatic disease and died after 8 months. The course of epithelioid sarcoma in this site seems to be more aggressive than in typical extremity locations and was consistent with other cases described in the literature.