Uveal malignant melanoma and optic nerve glioma in von Recklinghausen's neurofibromatosis.
A case of uveal malignant melanoma and contralateral optic nerve glioma is described in a 53-year-old Caucasian male with multiple uveal melanocytic hamartomas and neurofibromatosis. The eye was enucleated, and histologically the melanoma was found to consist of 70% epithelioid cells, with many bizarre, multinucleated forms. CT scan demonstrated a non-enhancing, fusiform enlargement of the contralateral optic nerve with enlargement of the optic canal and intracranial extension. This combination of tumours has not previously been reported in a patient with neurofibromatosis and serves to emphasise the common neuroectodermal origin of tumours in this autosomal dominant condition.
Antle
C M
CM
Department of Pathology, Vancouver General Hospital, British Columbia, Canada.
Damji
K F
KF
White
V A
VA
Rootman
J
J
D003390:Cranial Nerve Neoplasms; D005910:Glioma; D006801:Humans; D008297:Male; D008545:Melanoma; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009456:Neurofibromatosis 1; D009901:Optic Nerve Diseases; D014604:Uveal Neoplasms