Uveal malignant melanoma and von Recklinghausen's neurofibromatosis

A case of uveal malignant melanoma with extensive spread to the central nervous system occurring in a 63-year-old woman with peripheral-type von Recklinghausen's neurofibromatosis was studied by light microscopy. The tumor histologically consisted of large, loosely coherent epithelioid cells with abundant eosinophilic cytoplasm and large, irregular nuclei with prominent nucleoli. The tumor cells contained variable amounts of melanin pigment and exhibited positive immunohistochemical staining for S-100 protein and neuron-specific enolase. A review of the literature disclosed only ten previous cases of uveal malignant melanoma associated with von Recklinghausen's neurofibromatosis. A predominance of women and of the peripheral variant of von Recklinghausen's neurofibromatosis was noticed in the cases reported.