Journal of Neuro-Ophthalmology, September 2014, Volume 34, Issue 3

Vitreous Seeding From a Large Optic Disc Melanocytoma

Vitreous Seeding From a Large Optic Disc Melanocytoma Haoyi Guo, MMed, Yujun Li, MMed, Zhao Chen, MMed, Xirang Guo, BMed Abstract: We report the case of a 17 year-old man with a large optic disc melanocytoma that underwent spontane-ous rupture and seeding of the vitreous with pigmented cells. Potential pathogenic mechanisms and visual progno-sis of this rare event are discussed. Journal of Neuro-Ophthalmology 2014;34:276-277 doi: 10.1097/WNO.0000000000000134 © 2014 by North American Neuro-Ophthalmology Society Optic disc melanocytoma (ODMC) is a rare, benign intraocular tumor that primarily occurs in the optic nerve head and manifests as a melanotic mass. It generally does not affect vision, although a small proportion of pa-tients may experience minor visual impairment as the tumor expands. On rare occasions, it may lead to severe vision loss (1,2). Our patient developed a dramatic fundus appearance because of vitreous seeding of pigmented cells but fortu-nately retained normal visual acuity. CASE REPORT A 17-year-old man reported a "fluttering shadow" in front of his right eye for 3 weeks. Five years previously, he was found to have a melanocytoma in his right eye (Fig. 1). At that time, his visual function was normal, and he had reg-ular follow-up examinations. At the time of presentation, the patient's visual acuity was 20/20, right eye and 20/15, left eye. Anterior segment examination was normal bilaterally, and intraocular pres-sures were 15 mmHg, right eye, and 16 mmHg, left eye. Although the left fundus was unremarkable, his right fundus showed disseminated pigmented cells into the vitreous cavity (Fig. 2A). This was confirmed with the findings on fluorescein angiography (Fig. 2B). Optical coherence tomog-raphy (Fig. 2C) and ultrasonography (Fig. 2D) demonstrated a posterior vitreous detachment in the right eye. The patient declined fine needle aspiration biopsy or vitrectomy and re-mained stable over 1 year of follow-up. DISCUSSION Although ODMC generally follows a benign clinical course, complications may occur during long-term follow-up. Approximately 4% of cases demonstrate vitreous seeding of pigmented cells (1), and various mechanisms have been proposed. One possibility is spontaneous tumor necrosis. Font et al (2) described a patient with a large ODMC, with massive dispersion of pigmented cells into the vitreous 19 years after initial evaluation. The patient developed cataract and glaucoma and ultimately lost all sight in the affected eye. The authors speculated that the pathomechanism involved phagocytosis of disseminated necrotic melanocytes and subsequent inflammation. Another potential mechanism is tumor invasion of the retina and release of pigmented cells into the vitreous. Mazzuca et al (3) reported 2 such patients, both associated with increase in size of the ODMC, and in 1 patient, there was dilation and tortuos-ity of the retinal venous system, suggestive of vascular compression by the tumor mass. A final potential mecha-nism is spontaneous vitreous detachment. This is the likely cause in our patient and is supported by the OCT and ultrasonographic findings (Figs. 2C, 2D). With disruption of the internal limit membrane, melanocyte cells dissem-inated into the vitreous cavity. Shukla et al (4) described a patient with pigment dispersion from an ODMC and severe loss of vision after 33 years of follow-up. The eye was ultimately enu-cleated, and pathologic results revealed malignant trans-formation of the tumor melanocytes. Our patient Department of Ophthalmology, Henan Eye Institute, Henan Eye Hospital, Zhengzhou, China. The authors report no conflicts of interest. Address correspondence to Haoyi Guo, MMed, Henan Eye Institute, Henan Eye Hospital, 7 Weiwu Road, Zhengzhou 450003, China; E-mail: [e-mail] 276 Guo et al: J Neuro-Ophthalmol 2014; 34: 276-277 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. declined vitreous biopsy, but he has retained normal visual acuity and intraocular pressure with a stable appear-ance of the ODMC. Nevertheless, careful follow-up of our patient is warranted for this rare complication of ODMC (5). REFERENCES 1. Shields JA, Demirci H, Mashayekhi A, Eagle RC, Shields CL. Melanocytoma of the optic disk: a review. Surv Ophthalmol. 2006;51:93-104. 2. Font RL, Chaques-Alepuz V. Giant optic disk melanocytoma complicated with massive intraocular seeding. Arch Soc Esp Oftalmol. 2011;86:158-161. 3. Mazzuca DE Jr, Shields CL, Sinha N, Bianciotto CG, Fox G, Shields JA. Progressive retinal invasion and vitreous seeding from optic disc melanocytoma. Clin Experiment Ophthalmol. 2012;40:e123-e125. 4. Shukla SY, Shields JA, Eagle RC, Shields CL. Transformation of optic disc melanocytoma into melanoma over 33 years. Arch Ophthalmol. 2012;130:1344-1347. 5. Lauritzen K, Augsburger JJ, Timmes J. Vitreous seeding associated with melanocytoma of the optic disc. Retina. 1990;10:60-62. FIG. 1. A. Melanocytoma of the right optic disc. B. On fluorescein angiography, the tumor shows hypofluorescence. C. Optical coherence tomography reveals a thin, echogenic line delineating the anterior aspect of the melanocytoma with complete obscuration of the optic disc. D. With ultrasonography, the tumor appears as a hyperechoic mass on the surface of the optic disc. FIG. 2. Fundus appearance (A) and fluorescein angiographic results (B) after vitreous seeding. There is evidence of vitreous traction on the tumor with optical coherence tomography (C), and a posterior vitreous detachment is seen on ultrasonography (D). Guo et al: J Neuro-Ophthalmol 2014; 34: 276-277 277 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.