Malignant rhabdoid tumor of the vulva: Case report and review of the literature with emphasis on clinical management and outcome

Background. Malignant extrarenal rhabdoid tumors (ERTs) of the vulva are rare but aggressive neoplasms. We describe the eighth reported case and the first to have long-term survival despite initial rapid recurrence. Case. A 40-year-old woman presented with a painless lump on the mons pubis. It was excised and revealed an ERT. One month later, the tumor had regrown. A wide local excision and superficial right groin node dissection was performed. Local recurrence was noted 11 months after the initial excision and was treated by wide excision and radiotherapy. She is without evidence of disease 61 months after initial presentation. Conclusion. A review of previously reported cases of ERT reveals that the median survival is 9 months. Local recurrences are common and the appearance of distant metastases is almost invariably rapidly fatal. Chemotherapy and radiotherapy do not appear to be effective in controlling disease. Initial surgical extirpation may offer the best chance of cure. (C) 2001 Academic Press.