Hyperplasia of the pigmented ciliary epithelium or the RPE usually develops in response to trauma, inflammation, or other ocular insults (Fig 17-14A). Because of their location, ciliary body lesions often do not become evident clinically. Occasionally, however, they may become large enough to simulate a ciliary body melanoma. Posteriorly located lesions may be more commonly recognized and can lead to diagnostic uncertainty. In the early management of these atypical lesions, observation to document their stability is often appropriate. In rare cases, adenomatous hyperplasia may mimic a choroidal melanoma.
Figure 17-14 Lesions of the RPE. A, Reactive hyperplasia. B, Simple hamartoma. C, Clinical appearance of a peripapillary combined hamartoma of the retina and RPE. Note obscuration of the retinal vessels in the superior aspect of the lesion, moderate deep pigmentation, and secondary hard exudates. D, Fluorescein angiogram of the lesion shows the vascular component of the hamartoma, composed of small capillary-like telangiectatic vessels. Note the relative hypofluorescence superior to the optic nerve head, which is due to the RPE component of this lesion.
(Parts A and B courtesy of Tero Kivelä, MD; parts C and D courtesy of Robert H. Rosa Jr, MD.)
Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.