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Departments of Neurology (Drs. Balmaceda, Fetell, and O'Brien), Neuropathology (Dr. Powers), and Neurosurgery (Dr. Housepian), Columbia Presbyterian Medical Center, New York Neurological Institute, New York, NY.
Two patients with congenital nevus of Ota developed intracranial malignant melanocytic tumors. One had a localized tumor that resembled a melanocytoma, but the other had a more highly malignant tumor that diffusely seeded the leptomeninges. There are 10 prior cases in the world literature. These cases are contrasted with the other disorders in which melanotic skin lesions are associated with CNS melanocytic tumors, including neurocutaneous melanosis, cellular blue nevus, and metastatic malignant melanoma. Each disorder tends to involve particular sites of the CNS. The nevus of Ota can be considered a neurocristopathy and, rarely, may give rise to malignant CNS lesions.
Address correspondence and reprint requests to DY. Michael R. Fetell, New York Neurological Institute, 710 W. 168th Street, New York, NY 10032.
Supported in part by the Roberta F. Sloate Neuro-oncology Research Fund.
Received September 9, 1991. Accepted for publication in final form June 17, 1992.
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