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.
Shields et al. (Tue,) studied this question.