Retina. 2026 Jul 1;46(7):1160-1169. doi: 10.1097/IAE.0000000000004827.
Summary
RPE tumors: Large clinical series including CHRPE (most common), torpedo, RPE hamartoma, combined hamartoma, adenoma, and adenocarcinoma — Retrospective, 926 patients.
Abstract
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 five-decade period.
Results: Of 926 consecutive patients with RPE tumors, the specific diagnosis included solitary congenital hypertrophy of the retinal pigment epithelium (n = 727, 79%), multifocal congenital hypertrophy of the RPE (n = 42, 4%), torpedo maculopathy (n = 7, 1%), RPE hamartomas associated with familial adenomatous polyposis (n = 10, 1%), congenital simple hamartoma of the RPE (n = 5, 1%), combined hamartoma of the retina and RPE (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 (P < 0.01), and prevalence of macular epiretinal membrane, cystoid macular, edema, and subretinal fluid on optical coherence tomography (P < 0.01). By autofluorescence and fluorescein angiography, nearly all lesions that were imaged were hypoautofluorescent/hypofluorescent except for combined hamartoma of the retina and RPE (P < 0.01). Outcomes revealed visual acuity loss ≥3 lines (≥15 letters) at 10 years more often in combined hamartoma of the retina and RPE (20%), adenoma (21%), and adenocarcinoma (100%) (P < 0.01) and 10-year nodular growth in congenital hypertrophy of the RPE (1%), adenoma (9%), and adenocarcinoma (100%) (P < 0.01).
Conclusion: Retinal pigment epithelial tumors comprise a spectrum in demographics, clinical features, and outcomes. Most remain stable over time with little impact on visual acuity except for combined hamartoma of the retina and RPE, adenoma, and adenocarcinoma.
