Retinal pigment epithelial hamartoma--unusual manifestations.

Abstract

SUMMARY Hamartoma of the retinal pigment epithelium is an uncommon tumour of young adults. We have seen 2 patients with this clinical diagnosis, both with unusual manifestations. In one patient growth of the tumour was observed over a 5-year period. In the second patient arterialarterial anastomoses were detected at a site distal to the tumour.Retinal pigment epithelial (RPE) hamartomas are rare tumours that are classically described as having no growth potential.' They have on occasion been misdiagnosed as juxtapapillary choroidal melanomas, especially in the small percentage ofRPE hamartomas that were believed to show growth.2 We have recently seen an adolescent with an RPE hamartoma showing photographic and angiographic evidence of marked growth. We also present a patient with an RPE hamartoma associated with arterial-arterial anastomoses in a site distal to the tumour.

Things that go bump in the light. The differential diagnosis of posterior uveal melanomas

“…In exceptional cases slow progressive enlargement may be observed. 130 The lesion is almost always unilateral; to date the author is only aware of seven reported cases of bilateral involvement. [131][132][133][134][135][136][137] Whilst most cases of CHR-RPE appear to be sporadic, there is increasing evidence that these hamartomas may be associated with both neurofibromatosis types 1 and 2 (NF-1, NF-2).…”

Combined Hamartoma of the Retina and Retinal Pigment Epithelium of the Juxtapapillary Retina and Optic Disc (With 1 color plate)

“…Gass [3]. Kojima et al [ 10], and Kozima and Futa [12], Although growth of tumor, arterial-arterial anastomoses, and vitreous hemorrhage secondary to hamarto ma have been previously demonstrated [16][17][18], these features were not found in any Japanese patient. Normal ERG responses and an abnormal ly low EOG light rise in the affected eye have been previously reported [7,10,12].…”