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.
Cancer 63:2216-2219, 1989.
ALIGNANT SCHWANNOMA (MS), neurofibrosar-M coma, or malignant neurilemoma, is a spindle cell tumor thought to originate from Schwann cells or from peripheral nerves. ' These rare tumors are often associated with von Recklinghausen's neurofibromatosis (NF),* but have also been reported in patients without that di~ease.~ They usually arise in the extremities, but have been seen, less commonly, in the trunk, head, or neck. Although rare cases of benign neurofibroma of the clitoris have been reported in children4-" and adults"." with NF, there are no documented cases of MS of the clitoris. We report on the clinical, pathologic, and therapeutic data of MS of the clitoris in a 1-year-old patient with MS.
Case Report
A I-year-old white girl with a previous diagnosis of von Recklinghausen's NF was brought to our attention having a 7-month history of an enlarging clitoris. Physical evaluation revealed a mild ptosis of the left eyelid, pseudoarthrosis of the left tibia, numerous cafe-au-lait spots on the trunk and legs, and a 4 X 2 cm firm, nontender, venous engorged clitoris (Fig. I). Laboratory studies, including serum testosterone and cortisol levels, were normal. Cytogenetic studies of peripheral blood revealed a 46 XX female chromosome karyotype. Radiographic studies of the head and computerized tomography of the head, thorax, ab-From the