Abstract
Purpose :
The clinical diagnosis and treatment of adenoma and adenocarcinoma of the retinal pigment epithelium (RPE) continues to be a challenge for ophthalmologists. The majority of published reports consist of 1-3 cases and little consensus exists in terms of diagnosis and treatment vs. observation. The purpose of this study was to describe the clinical and imaging characteristics, treatment options and pathologic features of 51 consecutive cases of adenoma and adenocarcinoma of the RPE and to examine when observation or treatment is favorable.
Methods :
Retrospective study of 51 patients diagnosed with adenoma or adenocarcinoma of the RPE at the Ocular Oncology Service of Wills Eye Hospital, Philadelphia between October 1980 and February 2018. Referring diagnosis, demographic and clinical features, and treatment modality were documented. Multimodal imaging, cytologic and histopathological features were assessed.
Results :
Mean age was 51 years (range 18-83) and most common referring diagnosis was choroidal melanoma 19/51 (37%). Visual acuity (VA) was ≥20/40 (n=30, 59%), 20/50-20/150 (n=7, 14%) and ≤20/200 (n=14, 27%). Mean tumor thickness and diameter were 3.5 mm and 6.1 mm, respectively. Most lesions appeared black 28/51 (55%) and abruptly elevated 42/51 (82%). Feeder vessels (n=31/51, 61%), lipid exudation (n=28/51, 55%), basal congenital hypertrophy of the RPE (n=11/51, 22%) and exudative retinal detachment (n=11/51, 22%) were observed. Primary treatments included observation 29/51 (57%), partial lamellar sclerouvectomy 9/51 (18%), enucleation 5/51 (10%), plaque radiotherapy 3/51 (6%), intravitreal anti-vascular endothelial growth factor or corticosteroids 3/51 (6%) and laser photocoagulation 2/51 (4%). Mean follow-up was 82 months (n=35, range 0.3-354). Final VA was stable or improved in 17/32 (53%). Tumor growth was observed in 12/35 (34%) and eye retention rate was 32/39 (82%).
Conclusions :
RPE adenoma or adenocarcinoma can be identified by clinical and imaging characteristics. Observation is preferred in asymptomatic patients, and treatment for progressive lesions should be individualized.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.