Coexistence of 3 tumors of neural crest origin: neurofibroma, meningioma, and uveal malignant melanoma.

OBJECTIVE To describe the clinical findings in a patient who developed a neurofibroma, meningioma, and choroidal melanoma. METHODS Clinical and histopathological findings of the case are reviewed and presented. RESULTS The patient had a right superolateral periorbital neurofibroma, a right sphenoid wing meningioma, and a left choroidal juxtapapillary malignant melanoma. All 3 tumors are derived from neural crest cells. CONCLUSIONS To our knowledge, this is the first report of a patient with this combination of 3 neural crest-derived tumors. This case is most appropriately classified as a complex neurocristopathy, a disorder involving the aberrant and pathological proliferation of multiple tissues derived from neural crest cells.