Rare case of proximal epitheloid sarcoma of vulva in a young adult – Case report and review of literature

Vulval tumors are rare disease and account for 4% of gynaecological malignancies. (Newman and Fletcher, 1991) These rare tumors are disease of elderly age group. Primary vulval sarcomas account for 1-3% of vulval malignancies (Newman and Fletcher, 1991; Disaia et al., 1971; Curtin et al., 1995). The most common subtype is leiomyosarcoma of vulva. Few subtypes of vulval sarcomas are seen in adolesents and young adults. One of such rare type vulval sarcoma is epitheloid sarcoma. The epitheloid sarcoma variety of soft tissue sarcoma is seen in proximal and distal extremities of body. The proximal type tumors are found to be more aggressive with poor prognosis. The mean age of presentation is 36 years, with patients as young as 17 years are also been reported. We report a case of proximal type of epitheloid sarcoma of vulva with multiple recurrence of tumor managed with surgery and chemotherapy.