Optical coherence tomography angiography of a pigmented Fuchs' adenoma (age-related hyperplasia of the nonpigmented ciliary body epithelium) masquerading as a ciliary body melanoma

Abstract

PurposeTo report a case of age-related hypertrophy of the nonpigmented ciliary epithelium (ARH-NPCE) clinically resembling a ciliary body melanoma and report the optical coherence tomography angiography (OCTA) findings associated with this lesion.ObservationsA 51-year-old male was referred for evaluation of a deeply pigmented ciliary body mass with extension through the iris root. Iridocyclectomy was performed due to concern for ciliary body melanoma. Histopathologic analysis was instead consistent with ARH-NPCE, also known as Fuchs' adenoma. Prior to surgery, OCTA images revealed abnormal vasculature in the area of the lesion. Vessels in the peripheral iris approaching the lesion appeared more tortuous and were non-radial as compared with normal iris vessels. The ciliary body mass itself could not be penetrated using an OCTA system operating at 1050 nm.Conclusions and importanceARH-NPCE may clinically resemble a pigmented ciliary body melanoma. This is the second case describing this clinical scenario, which may be more common than previously thought. Non-invasive imaging with OCTA revealed an abnormal peripheral iris vasculature pattern in the area of the iridociliary mass characterized by disorganized, tortuous, and non-radial vessels. Despite advances in longer wavelength OCTA systems, poor penetration of the ciliary body lesion precluded imaging of the intratumoral vessels in this location.

Fuchs-Adenom ungewöhnlicher Ausprägung: Maskerade eines Ziliarkörpermalignoms

“…Bisher wurde nur in wenigen Fällen von einem Fuchs-Adenom mit einer dem unseren Fall vergleichbaren Größe berichtet (je nach Bericht 3 mm Dicke bzw. 1,8 mm Höhe, 2,7 mm längs und 2,8 mm quer)[4,5]. Ein begleitendes Hyphäma wurde unseres Wissens beim Fuchs-Adenom bisher nicht beobachtet.…”