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Richard K Lee, Elaine Han, Peng Lu, Jinrui Ma, Pedro Monsalve, Sander Dubovy; Clinicopathologic Features of Rare Non-Pigmented Ciliary Body Adenomas: a case series and literature review. Invest. Ophthalmol. Vis. Sci. 2019;60(9):712.
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© ARVO (1962-2015); The Authors (2016-present)
To present two cases of rare ciliary body adenomas and compare and review these with the current literature
A retrospective review of two non-pigmented ciliary body adenomas reviewed at the Bascom Palmer Eye Institute’s Florida Lions Eye Bank Ocular Pathology Laboratory. Histopathology and immunostaining is presented along with clinical history and ocular imaging including magnetic resonance imaging (MRI), ultrasound biomicroscopy (UBM), and anterior segment optical coherence tomography (OCT) imaging.
Both patients presented with ciliary body masses with subsequent ciliary body tumor resection. Tumor 1 was from an 8-year-old male presenting with a 1 year history of progressive vision loss and 1 month history of conjunctival congestion, corneal edema, keratic precipitates, and pain in the right eye associated with a large cystic lesion filling almost 1/3 of the superior and temporal anterior chamber adherent to the iris. Visual acuity was reduced by corneal edema, the mass blocking the visual axis, and inflammatory glaucoma. Tumor 2 was a 42-year-old female presenting with a mass in the left eye on the ciliary body, as well as an iris nodule, which was biopsied and subsequently excised.Both lesions exhibited clumped epithelium which contained foci of PAS-positive basement membrane between islands of cells. The cells contained variably clear cytoplasm with round nuclei and small inconspicuous nucleoli without marked atypia or pleomorphism. Alcian Blue stain is positive between islands of cells. KI-67 proliferation marker reactivity was minimal (less than 1%) in both tumors. Bother tumors were also S-100 positive. Immunostains for Tumor 1 were negative for chromogranin, SALL-2, and TTF-4.
Non-pigmented ciliary body adenoma is a rare tumor that is treated by block excision for a cure. In order to make the diagnosis of NPCE adenoma, resection and histopathological identification are necessary, especially since these tumors have a variety of clinical presentations and can be confused with other tumors, such as ciliary body melanoma. Immunohistochemial results have shown consistency in S-100 positivity. Despite the benign classification of these tumors, severe ocular complications commonly affecting visual acuity and causing secondary cataracts, intra-ocular inflammation, and secondary glaucoma have been reported in a review of the literature.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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