Malignant schwannoma of the clitoris in a 1-year-old child.

A 1-year-old infant with von Recklinghausen's neurofibromatosis was seen because of increasing clitoral enlargement over a 7-month period. The mother, who also had neurofibromatosis, was treated 2 years beforehand for an acoustic neuroma. The child was treated by radical clitorectomy. Pathologic examination revealed malignant schwannoma, a tumor not previously described in this site. Vincristine, dactinomycin, and cyclophosphamide chemotherapy was given for 2 years to prevent local recurrence and metastatic spread. The child remains tumor free 2.5 years after diagnosis.