Familial aggressive nevi of the iris in childhood

We report the occurrence of amelanotic melanocytic tumors of the iris arising in the second decade of life in two successive generations. Clinically, both tumors enlarged rapidly, with subsequent enucleation of the eye in one patient. In both cases, histologic examination revealed an aggressive iris nevus composed of spindle A cells. Both lesions had similar argyrophilic nucleolar organizing region counts, which may be of prognostic significance. A possible autosomal-dominant pattern of inheritance for this rare tumor is discussed.