Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: A case report

Ewing's sarcomas/peripheral primitive neuroectodermal tumors (ES/pPNETs) are high-grade malignant neoplasms rarely found outside the skeletal system Only 12 cases of vulvar ES/pPNET have so far been reported, all involving children or women of child-bearing age. We describe the case of a 52-year-old woman who was admitted to our hospital for the local excision of a 4 cm vulvar mass, originally thought to be a Bartholin's gland cyst It was subsequently found to consist of small round cells positive for anti-CD99 antibody, thus suggesting a diagnosis of ES/pPNET The demonstration of EWSRI gene translocations by means of fluorescent in situ hybridization excluded small-cell carcinoma, squamous cell carcinoma of the small type, Merkel cell carcinoma, and lymphoblastic lymphoma. After surgery, the patient received six cycles of polychemotherapy and radiotherapy: she is still alive and well after 1 year of follow-up. Our findings underline the crucial role of molecular biology techniques in the differential diagnosis of small round cell tumors in these unusual locations (C) 2009 Published by Elsevier GmbH