Vitreous seeding associated with melanocytoma of the optic disc

The authors describe an unusual case of melanocytoma of the optic disc associated with seeding of extracellular pigment or melanin-containing macrophages into the overlying vitreous. The possible mechanisms responsible for this clinical picture are discussed.     

视盘黑色素细胞瘤多模态影像学特征的临床研究

目的探讨视盘黑色素细胞瘤(MCOD)的多模态影像学特征。方法收集2010年8月至2022年3月就诊于首都医科大学附属北京同仁医院眼科中心确诊为MCOD患者21例(21只眼)的临床资料进行研究。其中,男性4例(4只眼),女性17例(17只眼),年龄28~82岁,平均年龄(53.1±14.6)岁。分别进行了彩色眼底照相、眼底自发荧光(FAF)、近红外反射成像(NIR-I)、荧光素眼底血管造影(FFA)、吲哚菁绿血管造影(ICGA)、频域光学相干断层扫描(SD-OCT)及光相干断层扫描血流成像(OCTA)检查,观察肿瘤的颜色、大小、位置、边界、是否继发视盘改变、与邻近视网膜或脉络膜的关系、肿瘤FAF特征、NIR-I特征、肿瘤荧光特征、视盘荧光特征、肿瘤的SD-OCT分型及OCTA血流特征。均采用频数和百分比进行描述。结果彩色眼底照相检查中,MCOD呈墨黑色、颜色均匀且边界清晰者17例(17只眼),占80%(17/21)。瘤体位于视盘颞侧者10例(10只眼),占47.6%(10/21)。出现视盘继发改变者14例(14只眼),占66.6%(14/21)。蓝光FAF检测MCOD均表现为完全的低自发荧光,占100%(21/21)。NIR-I检测MCOD均表现为边界清晰的高反射病灶且瘤体表面均呈平行纤维样或绒毛样外观,占100%(21/21)。FFA和ICGA检查MCOD均为低荧光,FFA早期可发现瘤体表面的细小血管者16例(16只眼),占76.2%(16/21)。接受SD-OCT检查MCOD患者10例(10只眼),呈典型表现者7例(7只眼),占70%(7/10);瘤体的不同部位分别呈典型和非典型表现者2例(2只眼),占20%(2/10),呈非典型表现者1例(1只眼),占10%(1/10)。接受OCTA检查者4例(4只眼),均可检测到表面瘤体浅层和深层血管网,占100%(4/4)。结论多模态影像检查可以为MCOD的诊断提供重要的信息。MCOD在NIR-I和FAF检测中的特征性表现具有诊断和鉴别诊断价值。     

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

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 14 months’ 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 disc.     

Malignant transformation of an optic nerve melanocytoma

A 47-year-old woman who had had a flat, hyperpigmented lesion of the optic disc and juxtapapillary choroid for at least 17 years experienced acute visual loss. This was caused by rapid growth of a pigmented tumour emanating from the original lesion. Histopathological examination of the enucleated globe revealed a large malignant melanoma originating from a melanocytoma (magnocellular nevus) within the optic nerve and peripapillary choroid. This case represents a very rare instance of malignant transformation of a normally benign tumour.     

Malignant transformation of an optic disk melanocytoma

PURPOSE: To report a case of malignant transformation of an optic disk melanocytoma with a second melanocytoma in the ciliary body. METHODS: Clinical data including visual acuity, visual fields, color fundus photographs, fluorescein angiogram, and ultrasonogram and histopathologic studies of this case were reviewed. RESULTS: The right eye of a 65-year-old white woman was diagnosed with melanocytoma of the optic nerve. Four years later, the tumor became significantly larger. The best-corrected visual acuity declined from 20/40 to counting fingers and the size of the tumor increased fourfold in 2 years. The right globe was enucleated. Histopathologic studies demonstrated moderately pigmented spindle-B malignant melanoma cells adjacent to and within a population of large, polyhedral, heavily pigmented melanocytoma cells that extended to the lamina cribrosa and optic nerve. There was also a deeply pigmented melanocytoma in the ciliary body. CONCLUSION: This is a rare case of malignant melanoma transformed from an optic disk melanocytoma. Periodic follow-up of the patient with optic disk melanocytoma is necessary.     

视乳头色素细胞瘤的临床表现和随诊

目的 探讨视乳头色素细胞瘤的临床表现和随诊观察的结果.方法 回顾性系列病例研究.回顾分析1978至2007年间确诊的10例视乳头色素细胞瘤患者的临床资料.检在项目包括直接、间接眼底镜及裂隙灯前置镜查眼底,眼底照相,荧光素眼底血管造影(FFA),吲哚氰绿眼底血管造影(ICGA),相干光断层扫描(OCT)及B超扫描等.结果 10例患者中男性9例、女性1例,年龄30～58岁,平均43岁.10例均为单眼患病,左、右眼各5例.视力:光感2例,0.1者1例,其余7例均为1.0以上.1例有广泛视网膜下积液,B超扫描显示视乳头上有一肿物,经玻璃体细针吸取肿瘤组织后活检,诊断为良性色素细胞瘤.1例,在1年的观察中,肿瘤隆起增加,视野缺损扩大,邻近玻璃体色素颗粒增多,为除外恶性变,应患者要求摘除眼球.病理诊断为良性视乳头色素细胞瘤,肿瘤细胞生长活跃,但无恶性改变.其他为8例色素性隆起肿物,位于视乳头上或有部分越过边界,在随诊1.0～18.0年(平均3.5年)的观察中,视力未减退,肿瘤无变化.其中1例经FFA或ICGA检查发现为双侧脉络膜斑痣.结论 视乳头色素细胞瘤是眼内良性肿瘤,一般不影响视力但应长期随诊观察.     

Optical coherence tomography study of optic disc melanocytoma.

Optic disc melanocytoma is a well-known clinical entity occurring as a deeply pigmented lesion on the optic nerve head. A 40-year-old man with a pigmented lesion overlying the left optic disc was evaluated with optical coherence tomography. The single high reflectance band from the anterior edge of the disc lesion with optical back shadowing behind on optical coherence tomography helped to pinpoint the diagnosis of the lesion as optic disc melanocytoma. Quantitative assessment of the mass with optical coherence tomography for its growth did not show any increase in size horizontally or vertically after 6 months. Optical coherence tomography is useful for obtaining the exact measurements of the mass lesion and may be used as a tool to follow the progress and growth pattern of the lesion.     

Organized vitreous hemorrhage masquerading as an optic disc melanocytoma

PURPOSE: To present a case of organized vitreous hemorrhage masquerading an optic disc melanocytoma. DISCUSSION: Optic nerve head melanocytoma is a benign slightly pigmented lesion arising from the edge of the disc presenting with a filed defect. Pigmented nature of the lesion in question due to presence of haemosidrin laden macrophages led a diagnostic dilemma. Trans vitreal biopsy confirmed the diagnosis. CONCLUSIONS: Althought classical in presentation organized blood clots can masquerade a number of lesions including a melanocytoma as in the present scenario.     

Central retinal vascular obstruction secondary to melanocytoma of the optic disc

A 35-year-old black man developed abrupt visual loss in his left eye. Ophthalmic examination revealed a deeply pigmented mass obscuring the optic disc, hemorrhagic retinopathy, and signs of central retinal vascular obstruction. Fluorescein angiography disclosed sluggish filling of the retinal blood vessels; ultrasonography disclosed an acoustically solid mass in the optic nerve head. Cytopathologic findings of a fine needle aspiration biopsy specimen demonstrated probable benign tumor cells, but melanoma could not be excluded. Histopathologic findings in the enucleated eye revealed a large, necrotic melanocytoma of the optic disc and hemorrhagic necrosis of the retina secondary to obstruction of the central retinal artery and vein. Melanocytoma of the optic nerve can undergo spontaneous necrosis and induce central retinal vascular obstruction. Abrupt visual loss in a patient with a melanocytoma does not necessarily imply malignant transformation.     

Angle closure glaucoma as initial manifestation of melanocytoma of the optic disc

A 44-year-old man had progressive visual loss and pain due to angle-closure glaucoma associated with a papillary pigmented lesion. The eye was enucleated because of intractable pain and the suspicion of a malignant tumor. Histologic and electron microscopic examination of the enucleated eye revealed a partially necrotic melanocytoma of the optic nerve. There was histologic evidence of vaso-occlusive disease within the optic nerve that resulted in ischemic necrosis of the tumor, hypoxic retinopathy and neovascular glaucoma. This report emphasizes that significant complications may occur in otherwise typical intraocular nevi.     

Optic disc melanocytoma

PURPOSE: Presentation of the cases of melanocytoma observed in the latest time in our clinic. MATERIAL AND METHODS: We present 2 cases - one in a 41 years old women S.M. and in a 77 years old women P.J. CONCLUSIONS: In case of melanocytoma there should be performed specialistic ancillary tests and the patients should be observed with photographic documentation.