Retinal pigment epithelial (RPE) tumors in 948 eyes of 926 patients

Purpose: To better define the frequency and types of retinal pigment epithelial (RPE) tumors. Methods: Retrospective review of all computer-coded RPE tumors over a 5-decade period. Results: Of 926 consecutive patients with RPE tumors, the specific diagnosis included solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE) (n=727, 79%), multifocal CHRPE (n=42, 4%), torpedo maculopathy (n=7, 1%), RPE hamartomas associated with familial adenomatous polyposis (RPEH-FAP) (n=10, 1%), congenital simple hamartoma of the RPE (CSHRPE) (n=5, 1%), combined hamartoma of the retina and RPE (CHRRPE) (n=99, 11%), benign RPE adenoma (n=34, 3%), and malignant RPE adenocarcinoma (n=2, <1%). There were differences in RPE tumors regarding patient age ( p <0.01), race ( p <0.01), sex ( p <0.01), presenting visual acuity ( p <0.01), number of tumors ( p <0.01), tumor basal diameter ( p <0.01), tumor thickness ( p <0.01), and distance to the optic disc ( p <0.01) and foveola ( p <0.01). There were differences in RPE tumors regarding imaging with ultrasonography ( p <0.01), optical coherence tomography (OCT) ( p <0.01), and prevalence of macular epiretinal membrane, cystoid macular, edema, and subretinal fluid on OCT ( p <0.01). By autofluorescence and fluorescein angiography nearly all lesions that were imaged were hypo-autofluorescent/hypo-fluorescent except for CHRRPE ( p <0.01). Outcomes revealed visual acuity loss ≥3 lines (≥15 letters) at 10 years more often in CHRRPE (20%), adenoma (21%), and adenocarcinoma (100%) ( p <0.01) and 10-year nodular growth in CHRPE (1%), adenoma (9%), and adenocarcinoma (100%) ( p <0.01). Conclusions: RPE tumors comprise a spectrum in demographics, clinical features, and outcomes. Most remain stable over time with little impact on visual acuity except for CHRRPE, adenoma, and adenocarcinoma.