Extraosseous Ewing sarcoma of the vagina: a rare entity

Ewing sarcoma, a highly malignant neoplasm of the bone, usually occurs during childhood. About 15% are extraosseous. The Ewing family of tumors (EFTs) are extremely rare in the vagina. A 40-year literature review from 1970 to 2010 revealed only nine cases. A 32-year-old woman presented with a painless vaginal mass. A wide excision was performed. Histopathology, immunohistochemistry and molecular studies confirmed extraosseous vaginal Ewing sarcoma. Despite aggressive chemotherapy with a good initial response, she developed local recurrence and metastasis to the spine and pelvis and succumbed 22 months later. A previous infiltrating ductal breast cancer, treated and in remission complicated the picture. We present the tenth case of vaginal Ewing sarcoma and the fourth to be confirmed by molecular studies. We stress the importance of molecular techniques in definitely diagnosing EFTs, especially those arising at unusual sites, particularly in the context of a previous diagnosis of breast cancer.