A woman in her 40s presented with a well-circumscribed, pigmented, episcleral lesion in her right eye (Figure, A). Results of ultrasound biomicroscopy showed an extension of a dome-shaped primary ciliary body tumor (Figure, B). Standardized A-scan echography showed medium internal reflectivity. To rule out a malignant neoplasm, a biopsy of the extrascleral tumor portion alone was performed, similar to the no-touch technique for conjunctival melanomas.1 Results of histopathologic examination revealed a well-circumscribed, pigmented, spindle cell tumor that had small nuclei with homogeneous chromatin distribution not suggestive of a malignant neoplasm, a lack of mitotic figures, and strong immunopositivity for melan A. Accordingly, the lesion was classified as a nevocytic ciliary body nevus with the exceptional feature of extrascleral extension, a finding predominantly appearing as a clinical feature of melanomas2 or very rarely of melanocytomas.3,4 Clinical and echographic follow-up revealed no sign of intraocular tumor growth or extraocular recurrence for 24 months. Best-corrected visual acuity remained 20/16 OD during the entire course.
Koch KR, Ortmann M, Heindl LM. Extraocular Extension of a Benign Ciliary Body Nevus. JAMA Ophthalmol. 2015;133(12):e153137. doi:10.1001/jamaophthalmol.2015.3137