Adenoma of the Ciliary Pigment Epithelium with Diffuse Iris Pigment Dispersion

Correspondence To the Editor: Adenoma of the ciliary pigment epithelium (CPE) is an exceedingly rare tumor that is difficult to clinically differentiate from malignant melanoma. [1] Herein, we report a case of an adenoma of the CPE in Chinese women and discuss the clinical features of this unusual tumor. A 57‑year‑old woman complaining of obviously visual deterioration vision in her right eye for 1‑year presented to Beijing Tongren Hospital in December 2013. There was no previous history of ocular trauma or intraocular inflammation. On examination, the visual acuity was 0.2 in the right eye and 1.2 in the left eye. Intraocular pressure readings were 23.8 mmHg and 14.6 mmHg in the right and left eyes, respectively. Slit‑lamp examination revealed diffuse fine pigmentosus keratic precipitates on the posterior corneal surface and diffuse pigment dispersion on the iris in the right eye. There was no visible iris neovascularization and iridodonesis. After mydriasis, a dark brown mass occupied the superotemporal posterior chamber, and a segmental cataract was present with amber color [Figure 1a]. The left eye was entirely normal. Bilateral fundus was normal by indirect ophthalmoscopy. Ultrasonography (US) showed an acoustically solid, abruptly elevated, hillock‑shaped mass, measuring 9.6 mm × 7.6 mm (basal) × 5.4 mm (apical thickness) [Figure 1b]; ultrasound biomicroscopy demonstrated a moderately irregular acoustically solid in 10:30–13:30 o'clock meridians in the ciliary body, measuring 9.89 mm × 6.96 mm × 4.57 mm. The lesion had invaded the structure of chamber angle and the root of the iris [Figure 1c]; magnetic resonance imaging (MRI) showed that the mass was hyperintense in T1‑weighted images and hypointense in T2‑weighted images to the brain [Figure 1d]. The tumor showed enhancement after gadolinium. The patient underwent local resection of the tumor in the form of iridocyclectomy. Histopathologically, the tumor showed heavy pigmentation and nests of pigmented epithelial cells forming gland‑like structures that were separated by vascularized fibrous connective tissue [Figure 1e]. They contained numerous characteristic round or oval clear vacuoles, encompassed by cells whose cytoplasm was replete with large, spherical melanosomes [Figure 1f]. No mitoses were seen. The tumor was diagnosed as adenoma of the pigmented ciliary epithelium. The CPE as a stretch from the retina pigment epithelium can sometimes undergo reactive proliferation and migration. However, true tumors of the pigment epithelia of the ciliay body are extremely rare. A patient with an adenoma of the CPE may be asymptomatic, or impairment of visual acuity …