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

Abstract

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.

Schwannoma of the clitoris

“…Our case was composed entirely of Type A. The malignant potential of Schwannoma is low, but cases of malignant schwannoma of the vulva have been reported (8–10).…”

Neurofibromatosis type 1 with external genitalia involvement

“…The bladder and lower ureters are most commonly affected. Most often, genital involvement is related to clitoral hypertrophy in girls with NF1 [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. However, neurofibroma involving male genitalia was reported earlier than in females [20].…”

“…Preoperative appearance of external genitalia in both patients showed a redundant and soft prepuce, but not an enlarged clitoris. Malignant schwannoma on microscopic examination has been reported in one patient with clitoris involvement [19].…”

Clitoromegaly in neurofibromatosis

“…A 25-month-old Caucasian girl was seen for congeni- Craven and Bresnahan, 1983Diekman et al, 1967Franceschini et al, 1978Greer and Pederson, Griebel et al, 1991Haddad and Jones, 1960Haraoka et al, 1988Hoffman, 1962Kaneti et al, 1988Kearse and Ritchey, Kenny et al, 1966Knudson and Amromin, Labardini et al, 1968 McKeown and Frazer, Messina and Strauss, 198119931961Nogita et al, 1990Nonomura et al, 1992Petsche and Radlinger, 1954Ravikumar and Lakshmanan, 1983Rink and Mitchell, 1983 Schepel and Tolhurst, Schreiber, 1963Thomas et al, 1989Van Buskirk et al, 1964Vitse et al, 1968 tal clitoromegaly that had slowly increased in size. Pregnancy and delivery were unremarkable.…”