Background: Melanocytoma of the optic disc is a benign melanocytic tumor that rarely causes visual impairment. We report a case of a melanocytoma of the optic disc with a decreased vision related to a peripapillary choroidal neovascular membrane (PCNVM) that was successfully treated by submacular surgery. Methods: A 45-year-old southern European patient had a melanocytoma of the optic disc in his left eye with vision of 20/100. Fluorescein angiography demonstrated a PCNVM impeding the fovea associated with a subretinal hemorrhage. Results: The patient underwent a complete vitrectomy and removal of the PCNVM. Subsequently, the subretinal hemorrhage disappeared and visual acuity improved to 20/25. Visual acuity remained good for a period of 14months' follow-up without any recurrence of neovascular membrane. Conclusions: Submacular surgery is a potentially effective treatment for large PCNVM associated with a melanocytoma of the optic dis

Bovey, Etienne

Tran, H.

Uffer, Sylvie

Zografos, Leonidas

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Graefe’s Arch Clin Exp Ophthalmol(2006) 244: 1367–1369 CASE REPORTDOI 10.1007/s00417-006-0270-1H. Viet TranEtienne H. BoveySylvie UfferLeonidas ZografosReceived: 1 November 2005Revised: 29 December 2005Accepted: 9 January 2006Published online: 8 March 2006# Springer-Verlag 2006Peripapillary choroidal neovascularizationassociated with melanocytoma of the opticdisc: a clinicopathologic case reportAbstract Background: Melanocyto-ma of the optic disc is a benignmelanocytic tumor that rarely causesvisual impairment. We report a caseof a melanocytoma of the optic discwith a decreased vision related to aperipapillary choroidal neovascularmembrane (PCNVM) that wassuccessfully treated by submacularsurgery. Methods: A 45-year-oldsouthern European patient had amelanocytoma of the optic disc in hisleft eye with vision of 20/100. Fluo-rescein angiography demonstrated aPCNVM impeding the fovea asso-ciated with a subretinal hemorrhage.Results: The patient underwent acomplete vitrectomy and removal ofthe PCNVM. Subsequently, the sub-retinal hemorrhage disappeared andvisual acuity improved to 20/25. Vi-sual acuity remained good for aperiod of 14 months’ follow-upwithout any recurrence of neovascularmembrane. Conclusions: Submacu-lar surgery is a potentially effectivetreatment for large PCNVM asso-ciated with a melanocytoma of theoptic disc.Keywords Melanocytoma . Opticnerve . Choroidal neovascularmembrane . Submacular surgery .HistopathologyIntroductionMelanocytoma is a variant of melanocytic nevus and ismost often localized in the optic disc, although it can occuranywhere in the uveal tract [5, 7]. Melanocytoma of theoptic disc is a benign melanocytic tumor that rarely causesvisual impairment, but it can cause an afferent pupillarydefect and a visual field abnormality [2, 4]. When visualloss occurs, it is more likely due to necrosis of the benigntumor and loss of optic nerve function or compression ofadjacent blood vessels producing retinal vein or arteryocclusion rather than to malignant transformation [8].Proliferation of choroidal neovascularization on the surfaceof these lesions has been described in a limited number ofcases and formerly constituted an indication for enucle-ation due to suspected melanoma [1, 6]. To our knowledge,this is the first case of peripapillary choroidal neovascularmembrane (PCNVM) associated with a melanocytoma ofthe optic disc that has successfully been treated bysubmacular surgery.Case reportA 45-year-old southern European patient was referred toour department for evaluation and treatment of a recentlydiscovered pigmented mass on the optic disc of the left eyethat had been causing a severe loss of visual function for1 month. Best corrected visual acuity of the affected eyewas 20/60. Slit lamp examination was normal and intra-ocular pressure was 16 mmHg in both eyes. Visual fieldshowed a peripapillary scotoma and a small superiortemporal defect. The lesion was located in the temporalsector of the optic nerve and had a choroidal extension, andan intraretinal component with a feathery margin, compat-ible with its location in the nerve fiber layer (Fig. 1a). Alarge subretinal neovascular membrane was situatedbetween the optic nerve and the fovea. On both fluoresceinand indocyanin green (ICG) angiography the neovascularmembrane extended from the optic nerve to half the surfaceof the fovea. The tumor’s thickness was 2.0 mm on B-scanultrasonography. The diagnosis of a presumed melanocy-H. V. Tran . E. H. Bovey (*) .S. Uffer . L. ZografosJules Gonin University Eye Hospital,15, Av. de France,1004 Lausanne, VD, Switzerlande-mail: Etienne.bovey@ophtal.vd.chtoma complicated by neovascularization phenomena wasretained and since there was a relatively good residualvisual acuity, a periodic observation was decided upon.Three months after the initial examination, visual acuity,the size, and the shape of both tumor and PCNVMremained unchanged.After 5 months of follow up, visual acuity decreased to20/100 and an increased size of the arcuate scotoma andperipapillary defect was observed. Fundus examinationshowed a large subretinal hemorrhage, and fluorescein andICG angiography revealed an enlarged extension of thePCNVM to the entire fovea (Fig. 1b). There was noposterior vitreous detachment. The tumor dimensionsremained stable. The extensive size of the PCNVM wasnot accessible to laser treatment as the surface to be treatedwould have impinged on the macula. Thus, we decided toperform surgical excision of the PCNVM.A three-part pars plana vitrectomy was performed byone of the authors (EHB). The retinotomy was made at thesuperior aspect of the neovascular complex, parallel to thenerve fiber bundle, and the neovascular membrane wasgrasped and removed in a single piece. The posteriorhyaloid was adherent to the subretinal hemorrhage and tothe surrounding retina. The histopathologic examinationshowed a single ingrowth stalk of a dense, vascularizedfibrous tissue, situated in the interface of a degeneratedretinal pigment epithelium and an atrophic, disorganizedgliotic retina (GFAP positive). Neovessels did not invadethe retina and Bruch’s membrane was not included in theneovascular complex (Fig. 2a,b). A large vascularizedcluster of densely pigmented polyhedral melanocytic cellswas present beneath and above the retinal pigmentepithelium. The nuclei were small and regular with noprominent nucleoli. No mitotic figures were seen (Fig. 2c).On immunohistochemistry, the cells stained positive forHMB45 and Melan-A (Fig. 2d).Fourteen months after surgery, visual acuity improved to20/25 and the visual field defect decreased (Fig. 1c). Theretina was attached, the dimensions of the tumor remainedstable and fluorescein angiography did not reveal anyrecurrence of the PCNVM.DiscussionDevelopment of choroidal neovascularization related tomelanocytoma is a rare phenomenon and it can lead tosevere loss of visual function. This situation can bedifferentiated from a malignant transformation by theconstant size of the tumor. We are unaware of previousreports of PCNVM related to a melanocytoma of the opticdisc that was successfully removed by submacular surgery.Peripapillary melanoma with disruption of the Bruch’smembrane can mimic a melanocytoma of the optic disc. Ithas one choroidal part and an extension into the vitreouscavity at the junction of the retinal pigment epithelium andthe optic disc. However, peripapillary melanoma withdisruption of the Bruch’s membrane would have anextended choroidal part associated with alterations of thepigment epithelium, lipofuscin deposit, and show as aFig. 1 a At presentation. Fundusphotograph: serous retinal detach-ment surrounded by lipid depositsoverlying the choroidal compo-nent of the tumor. Fluoresceinangiography, arterio-venous phase:extensive neovascular membraneon the surface of the choroidallocalization of the tumor. Indocya-nin green (ICG), late phase: diffu-sion of the dye beyond the limitsof the lesion. b Five months later,before surgery. Fundus photo-graph: large subretinal hemorrhage.Fluorescein angiography, arterio-venous phase: progression of theneovascular membrane invadingthe fovea. Venous phase: leakageof the dye. c Fourteen months aftersurgery. Fundus photograph: thedimensions of the tumor remainedstable. Fluorescein angiography,early and late phase: occlusionof the choroidal neovascularizationand absence of diffusion of thedye. Alterations of the retinal pig-ment epithelium. Reattachmentof the retina1368pinpoint on fluorescein angiography. The vitreous partwould be darker, more abruptly elevated, and more likelyto have associated exudative retinal detachment. Finally,because of the fast growth of the tumor, a retinal nerve fiberlayer infiltration of pigment is absent.The histopathology of uveal melanocytoma was clearlydefined by Juarez and Tso in 1980 [3]. Two types of cellswere recognized. Type 1, which generally predominates, isa larger polyhedral, densely pigmented cell, with a small,bland nucleus, occasionally showing a small nucleolus.Type 2 is a smaller, less pigmented spindle cell, with amore conspicuous nucleolus. Neither type shows mitoses.These two types of cells are generally intermingled. In ourcase, the bleached preparation of the tumor showed thetypically abundant cytoplasm and the small, bland nucleusof the type 1 cell.Because PCNVM usually begins well away from thefovea, patients suffering from these neovascular mem-branes typically remain asymptomatic until the extensionof the lesions themselves or associated subretinal fluids orblood begin to impinge on the fovea. In patients with goodresidual visual acuity, the laser required for the ablation ofthese large neovascular complexes close to the optic nerveand the fovea would destroy the maculo-papillar bundle,and endanger the optic nerve. Therefore, in selected casessubmacular surgery is a valuable therapeutic modality.Acknowledgement We would like to thank Fromhold Samuel forhis technical support. This work was not sponsored by anycommercial organization. Financial interest: none. Grant: none.Fig. 2 Histopathology. a Part(A) of the membrane was re-moved with the overlying retina;part (B) without retina hasfolded above part A during thehistologic preparation. Infoldedneovascular membrane with acluster of pigmented melano-cytic cells (asterisk) situatedbeneath and above the retinalpigment epithelium (arrow; HE;original magnification, ×4).b Fibrovascular membrane(CNV) situated between a de-generated retinal pigment epi-thelium (arrow) and a glioticretina (asterisk). (HE; originalmagnification, ×40). c Bleachedpreparation showing polyhedraltumor cells with bland nuclei.(HE; original magnification,×100). d Positive staining of thetumor cells for HMB45 (originalmagnification, ×100)References1. Howard GM, Forrest AW (1966)Melanocytoma of the disc complicatedby hematoma under retinal pigmentepithelium. Trans Am Acad OphthalmolOtolaryngol 70:281–2852. Joffe L, Shields JA, Osher RH, Gass JD(1979) Clinical and follow-up studiesof melanocytomas of the optic disc.Ophthalmology 86:1067–10833. Juarez CP, Tso MO (1980) An ultra-structural study of melanocytomas(magnocellular nevi) of the optic diskand uvea. Am J Ophthalmol 90:48–624. Osher RH, Shields JA, Layman PR(1979) Pupillary and visual field eval-uation in patients with melanocytomaof the optic disc. Arch Ophthalmol97:1096–10995. Reidy JJ, Apple DJ, Steinmetz RL,Craythorn JM, Loftfield K, Gieser SC,Brady SE (1985) Melanocytoma: no-menclature, pathogenesis, natural his-tory and treatment. Surv Ophthalmol29:319–3276. Rubin ML (1976) Disciform lesionoverlying melanocytoma simulatingprogression of choroidal melanoma.Trans Am Ophthalmol Soc 74:282–2947. Zimmerman LE (1965) Melanocytes,melanocytic nevi, and melanocytomas.Invest Ophthalmol 34:11–418. Zografos L (2002) Melanocytomes. In:Tumeurs intraoculaires. Rapport SFO(ed) Masson, Paris, pp 82–941369

Peripapillary choroidal neovascularization associated with melanocytoma of the optic disc: a clinicopathologic case report

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Peripapillary choroidal neovascularization associated with melanocytoma of the optic disc: a clinicopathologic case report

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