Congenital Hypertrophy the Most Common RPE Tumor, Research Finds

CHRPE, the most frequently diagnosed tumor in this study, is typically benign and asymptomatic but can cause vision problems if located near the macula. Photo: Julie Rodman, OD. Click image to enlarge. Congenital hypertrophy of the RPE (CHRPE) was the most common type of tumor found in a recent review of 926 participants with RPE cancers in a recent Philadelphia study; adenoma of the RPE was the least common.RPE tumors “are fairly uncommon,” the authors began in their paper on the story for Retina. They “comprise a spectrum of lesions” and “can impact visual acuity over time,” rendering the identification of them all the more urgent. The tumors examined in this paper were as follows: solitary CHRPE, multifocal CHRPE, torpedo maculopathy, RPE hamartomas associated with familial adenomatous polyposis (RPEH-FAP), congenital simple hamartoma of the RPE (CSHRPE), combined hamartoma of the retina and RPE (CHRRPE), benign RPE adenoma and malignant RPE adenocarcinoma.The medical records of 926 patients from Wills Eye Hospital were selected for the study, with data ranging from May 1975 to December 2024. The researchers collected demographic (age, race, sex) and ocular (best-corrected Snellen visual acuity and IOP) information from each patient, as well as details on their tumors, including “the number of RPE tumors per eye (single, multiple), quadrantic tumor location (macula, superior, inferior, nasal, temporal, diffuse), largest tumor basal diameter and thickness, distance of RPE tumor from the optic disc and foveola.” They analyzed tumor images using fundus photography and autoflorescence, ultrasonography, OCT and fluorescein angiography.RPE tumors were found most often in white patients (86%) and female subjects (59%); additionally, multifocal CHRPE and RPE hamartomas associated with familial adenomatous polyposis were found most often in both eyes rather than only one. The authors noted that different tumor varieties presented at different ages in patients, with combined hamartoma of the retina and RPE occurring as early as 13 years of age and RPE adenocarcinoma at the oldest mean age of 65 years.Touching on the study’s potential limitations, the authors mentioned that their data came “from a single-center, referral-based department and might not represent the true frequency of each RPE tumor,” and that “the majority of patients were Caucasian,” which may not represent the demographics of those affected by RPE tumors outside a clinical setting.Click here for the journal source. Shields C, Attaseth T, Chou Y, et al. Retinal pigment epithelial (RPE) tumors in 948 eyes of 926 patients. Retina. March 10, 2026. [Epub ahead of print]. This article was developed by the editorial staff in conjunction with experts in the field. In the process, AI may have been among the editorial tools used to meet the goals of human editors, who approved all content.