Proximal-type epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva: a case report, review of the literature, and an argument for consolidation

Abstract

Background: Vulvar sarcomas, which comprise less than 2% of vulvar malignancies, are a heterogeneous group of tumors. Sub-categorization of these tumors makes treatment strategies difficult to evaluate. "Proximal" type epithelioid sarcoma (PES) and malignant rhabdoid tumor (MRT) describe a rare subset of vulvar malignancies which share histologic features and an aggressive clinical course.

Case: We describe a case of vulvar PES/MRT in a 35 year old female treated with radical excision, inguinal-femoral lymphadenectomy, and adjuvant radiation who remains without evidence of disease 40 months after surgery. We review all English language reports of vulvar PES or MRT, and compare treatments and outcomes.

Conclusion: Vulvar PES/MRT is a rare disease attended by high rates of local and distant relapse. Radical excision remains the foundation of treatment. The role of adjuvant therapy, and particularly radiation, remains unclear but merits consideration.