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Coexistence of 3 Tumors of Neural Crest Origin
Neurofibroma, Meningioma, and Uveal Malignant Melanoma
Arch Ophthalmol. 1998;116:1241-1243.
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.
Ronald E. Warwar, MD;
John D. Bullock, MD;
Jerry A. Shields, MD;
Ralph C. Eagle, Jr, MD
From the Departments of Ophthalmology (Drs Warwar and Bullock) and Physiology and Biophysics (Dr Bullock), Wright State University School of Medicine, Dayton, Ohio; and the Departments of Ocular Oncology (Dr Shields) and Ocular Pathology (Dr Eagle), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa.
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