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 feature s, and prognosis of adenoma of the CPE and to determine clinical features t hat may differentiate it from ciliary body melanoma. Patients and Methods: A retrospective review was performed of medical chart s, photographs, and pathological features of patients with adenoma of the C PE 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 melano ma in 7 patients and cyst in 1 patient. The lesions were all solitary and u nilateral and ranged from 3 x 3 x 3 to 13 x 13 X 8 mm. Clinically, all tumo rs 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 transilluminat ion, fluorescein angiography, and ultrasonography showed patterns that were helpful in differentiation from ciliary body melanoma. Fine needle aspirat ion biopsy, performed in 3 patients, was an accurate diagnostic adjunct. Mi croscopic diagnosis was adenoma of the CPE in 7 cases in which tissue was a vailable. A consistent histopathologic feature was the presence of typical clear vacuoles within the tumor. One tumor invaded the sensory retina. Resu lts of immunohistochemical studies were consistent with a tumor of neuroect odermal origin. Conclusions: Adenoma of the CPE has characteristic features that may help d ifferentiate 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.