Melanocytoma (magnocellular nevus) of the ciliary body: Report of 10 casesand review of the literature

Purpose: To describe the characteristics of melanocytoma (magnocellular nev us) of the ciliary body, an unusual melanocytic tumor. Design: Retrospective, clinicopathologic case series. Methods: Ten melanocytomas of the ciliary body were examined histopathologi cally. Five tumors were also examined by transmission electron microscopy. The clinical histories were abstracted from the patients' medical records, We compared the clinical, histopathologic, and ultrastructural findings of our 10 cases with those previously reported in the literature. Main Outcome Measures: To define clinical and histopathologic parameters of this rare tumor. Results: A total of 40 cases were summarized, including our 10 and 30 previ ously reported cases. The mean age of the 40 cases was 47 years. Twenty-six patients were female (65%), fourteen were male (35%). No laterality was ob served. Eighty percent of the patients were white and 10% were black. The m ost common clinical presentation was an asymptomatic dark spot involving th e iris and ciliary body or the sclera, Involvement of chamber angle structu res, with or without intrascleral extension, was observed in 85% (34 of 40 patients); but only 12% (2 of the 17 patients for whom information about in traocular pressure and local extension was available) had elevated preopera tive intraocular pressure. By electron microscopy, two distinct cell types were observed. Conclusions: Melanocytomas can usually be distinguished from other pigmente d lesions of the ciliary body by histopathologic criteria, but may be diffi cult to differentiate before surgery from other pigmented ciliary body tumo rs, including malignant melanomas, adenomas, and adenocarcinomas of the pig mented ciliary epithelium. Most tumors can be managed conservatively by iri docyclectomy. (C) 2000 by the American Academy of Ophthalmology.