Warwar R E, Bullock J D, Shields J A, Eagle R C
Department of Ophthalmology, Wright State University School of Medicine, Dayton, Ohio 45429-3487, USA.
Arch Ophthalmol. 1998 Sep;116(9):1241-3. doi: 10.1001/archopht.116.9.1241.
To describe the clinical findings in a patient who developed a neurofibroma, meningioma, and choroidal melanoma.
Clinical and histopathological findings of the case are reviewed and presented.
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.
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.