Azam Zafarbakhsh, Fariba Behnamfar, Parvin Rajabi, Zahra Sheikhsoleimani, Maryam Nazemi
Epithelioid sarcoma (ES) is a rare malignant soft tissue tumor, with the proximal-type variant (PES) representing an uncommon and aggressive subtype. Vulvar sarcoma predominantly affects young women, with a mean age of approximately 38 years. We report a case of a 22-year-old G1L1 Asian woman presenting with a painless, solid mass in the right labia majora, without tenderness or surface ulceration. Surgical management consisted of a right hemi-vulvectomy with a 2 cm margin from the tumor. Histopathological evaluation confirmed the diagnosis of epithelioid sarcoma of the vulva, and the surgical margins were free of tumor involvement. Postoperatively, the patient was followed every three months for the first two years and annually thereafter. Over a ten-year follow-up period, no evidence of local recurrence or metastasis was observed, and the patient experienced two successful pregnancies during this time. This case highlights the importance of considering vulvar sarcomas in the differential diagnosis of nonspecific vulvar lesions to ensure early recognition, accurate diagnosis, and appropriate management.