Acquired adenoma of the nonpigmented ciliary epithelium: analysis of five cases

Adenoma of the non-pigmented ciliary epithelium (ANPCE) is extremely rare. The aim of this study is to present our experience in treating this rare entity and to determine the long-term surgical outcomes of local tumor resection (partial iridocyclectomy with lamellar sclerouvectomy) in five cases of ANPCE in China.

The medical data of four women and one man ranging in age from 28 to 46 years (median, 38 years) with ANPCE were reviewed retrospectively at the Zhongshan Ophthalmic Center of Sun Yat-sen University, China. All patients had received local tumor resection and the diagnosis was confirmed by histopathological examination.

All five cases experienced blurred vision. The right eye was affected in four cases and the left in one. Slit-lamp biomicroscopy revealed a nodular, non-pigmented, gray-white mass with a slightly irregular surface. Tumor invasion through the peripheral iris was observed in two patients, two patients had multiple iris or ciliary cysts, and one patient had a partially bloodstained cornea. All tumors transmitted light readily. Ultrasound biomicroscopy showed a middle- or hyper-echoic solid mass in the ciliary body. MRI examination showed the tumor hyperintense to vitreous on T1WI and hypointense to vitreous on T2WI. Local resection was performed in all cases. After a median of 5.0 years of follow-up, no tumor recurrence was present in any of the cases and all involved eyes were saved. The visual acuity improved seven lines in three cases, decreased one line in one case, and vision was lost in one case.

ANPCE often presents in adults as gray-white in color, with an irregular and sometimes multilobulated surface. The tumor transmits light well. Local resection of the mass generally provides the patient with useful vision. Recurrence after surgical removal is unlikely.