Adenoma of the ciliary body pigment epithelium: the 1998 Albert Ruedemann, Sr, memorial lecture, Part 1.

BACKGROUND Adenoma of the pigment epithelium of the ciliary body (CPE) is a rare neoplasm. Most reported cases have been misdiagnosed as ciliary body melanoma. OBJECTIVES To evaluate clinical features, management, pathological features, and prognosis of adenoma of the CPE and to determine clinical features that may differentiate it from ciliary body melanoma. PATIENTS AND METHODS A retrospective review was performed of medical charts, photographs, and pathological features of patients with adenoma of the CPE who were treated by the authors. RESULTS Of the 8 patients with adenoma of the CPE, 3 were male and 5 were female. Seven were white, and 1 was Asian. The mean age at diagnosis was 51 years (range, 8-73 years). The referring diagnosis was ciliary body melanoma in 7 patients and cyst in 1 patient. The lesions were all solitary and unilateral and ranged from 3 x 3 x 3 to 13 x 13 x 8 mm. Clinically, all tumors were gray to black, had abruptly elevated margins, and were dome shaped. Associated findings included secondary cataract (6 patients) and vitreous hemorrhage (1 patient). Results of ancillary studies such as transillumination, fluorescein angiography, and ultrasonography showed patterns that were helpful in differentiation from ciliary body melanoma. Fine needle aspiration biopsy, performed in 3 patients, was an accurate diagnostic adjunct. Microscopic diagnosis was adenoma of the CPE in 7 cases in which tissue was available. A consistent histopathologic feature was the presence of typical clear vacuoles within the tumor. One tumor invaded the sensory retina. Results of immunohistochemical studies were consistent with a tumor of neuroectodermal origin. CONCLUSIONS Adenoma of the CPE has characteristic features that may help differentiate it from ciliary body melanoma. In contrast to melanoma, it is generally darker and its margins are more abruptly elevated. Although it is benign cytologically, it can exhibit growth. If the diagnosis is suspected, removal of the tumor by local resection is advisable.