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Malignant schwannoma of the clitoris in a 1-year-old child
W J Thomas1, H E Bevan, D G Hooper
1Department of Pediatrics, Naval Hospital, San Diego, California 92134-5000.
Insights
A rare malignant schwannoma was diagnosed in a 1-year-old infant with neurofibromatosis. This tumor, previously undescribed in this location, was successfully treated with surgery and chemotherapy.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Genetics
Background:
- Neurofibromatosis (NF1) is a genetic disorder predisposing individuals to various tumors.
- Malignant schwannomas are rare but aggressive tumors arising from nerve sheath cells.
- Clitoral enlargement in infants can indicate underlying pathology requiring investigation.
Observation:
- A 1-year-old infant presented with progressive clitoral enlargement over 7 months.
- The infant's mother had a history of neurofibromatosis and acoustic neuroma.
- The mother's neurofibromatosis diagnosis raised suspicion for a genetic predisposition in the child.
Findings:
- Pathologic examination confirmed a malignant schwannoma of the clitoris, a novel finding.
- The infant underwent radical clitorectomy for tumor removal.
- Post-operative chemotherapy with vincristine, dactinomycin, and cyclophosphamide was administered for 2 years.
Implications:
- This case highlights the importance of considering rare tumor types in infants with neurofibromatosis.
- Early diagnosis and multimodal treatment (surgery and chemotherapy) are crucial for favorable outcomes.
- The successful management suggests potential therapeutic strategies for similar rare pediatric malignancies.
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.