Adenoma and Adenocarcinoma of the Retinal Pigment Epithelium: A Review of 51 Consecutive Patients

Abstract

To describe the clinical and imaging characteristics, pathologic features, and management options of retinal pigment epithelium (RPE) adenoma/adenocarcinoma. Retrospective case series. Fifty-one patients with RPE adenoma/adenocarcinoma. Treatment options for the patients included observation, partial lamellar sclerouvectomy (PLSU), enucleation, and others. Factors related to visual acuity (VA) outcomes (>2 Snellen lines loss, poor final VA [≤20/200], good final vision [≥20/40]), tumor growth, and need for enucleation. The mean patient age at diagnosis was 51 years, and the majority of patients were white (40/51, 78%) and female (34/51, 67%). Primary management included observation (29/51, 57%), PLSU (9/51, 18%), enucleation (4/51, 8%), or others (9/51, 18%). Outcomes revealed decreased VA (10/32, 31%), poor final VA (17/32, 53%), good final VA (11/32, 34%), tumor growth (12/25, 48%), and need for enucleation (7/51, 14%). By multivariable analysis, features predictive of decreased VA included increasing baseline tumor thickness (P= 0.01) and presence of vitreous hemorrhage (P= 0.05). Factors predictive of poor final VA included presence of exudative retinal detachment (P= 0.004), baseline VA 20/50 to 20/150 (P= 0.008), and baseline VA ≤20/200 (P= 0.01). Absence of feeding and/or draining vessel was predictive of good VA (P= 0.004). Tumor growth was associated with multiple treatments (P= 0.02). Increased tumor thickness (P= 0.03) and presence of exudative retinal detachment (P= 0.01) were predictive of enucleation. RPE adenoma/adenocarcinoma can simulate choroidal melanoma. Greater tumor thickness, vitreous hemorrhage, exudative retinal detachment, and poor baseline VA predict worse visual outcome and greater risk for enucleation.