Congenital Hypertrophy of Retinal Pigment Epithelium With Overlying Thin Adenoma

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A 61-year-old white woman was found to have a sharply demarcated, pigmented lesion of the retinal pigment epithelium (RPE) at the equator of her right eye, surrounded by a pigmented and nonpigmented halo, consistent with congenital hypertrophy of the RPE (CHRPE). The mass measured 5 mm × 5 mm in basal dimension. Centrally, a more darkly pigmented slightly thickened area was observed (Figure 1A), measuring 0.4 mm in thickness by B-scan ultrasonography and 313 μm on optical coherence tomography (OCT). By OCT, the central portion had irregular optically dense thickening of the RPE with shadowing, more consistent with thin adenoma arising in CHRPE (Figure 1B).
Previous studies1 have shown that CHRPE can grow slowly over years and can develop RPE adenoma within the mass.2 Also, RPE adenoma is a slow growing neoplasm, often arising in CHRPE, and occasionally producing subretinal fluid with lipid exudation and remote macular edema.2,3 Result of OCT of CHRPE shows thickened, hyperreflective RPE with overlying photoreceptor loss.1,4,5 By OCT, adenoma demonstrates an elevated optically dense mass or thickening, which is more pronounced than the RPE thickening associated with CHRPE.3 Other OCT features of RPE adenoma include retinal invasion (Figure 1B) and dense posterior optical shadowing.3
In this case, the thin adenoma was followed conservatively as there were no visual-threatening findings. Over 12 months follow-up, the lesion has remained stable.
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