RETINAL PIGMENT EPITHELIAL TUMORS IN 948 EYES OF 926 PATIENTS.

Publication/Presentation Date

7-1-2026

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.

Volume

46

Issue

7

First Page

1160

Last Page

1169

ISSN

1539-2864

Disciplines

Medicine and Health Sciences

PubMedID

42308469

Department(s)

Medical Education

Document Type

Article

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