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Melanocytoma of the optic disk: a revie

Optic disc melanocytoma is a benign lesion of the optic nerve head that poses little threat to vision and carries low malignancy potential. 1-4 Although the color fundus photo showed a black color, the multicolor image detected the superficial nature of the lesion by showing a greenish hue along with the black color 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 Melanocytoma is a rare bening pigmented neoplasm, that usually affects anywhere in the uvea and can affect the optic nerve head, even involves the adjacent retina and choroid in some cases. Photomicrograph and cytologic features of optic disk melanocytoma (hematoxylin-eosin). Shields JA, Demirci H, Mashayekhi A, Eagle RC Jr, Shields CL • Melanocytoma can be located anywhere within the uveal tract but is most commonly found within the optic disc. • Although usually asymptomatic, optic disc melanocytoma can occasionally be associated with visual field defect, relative afferent pupil defect, visual loss, and pain

Optic Nerve Melanocytoma » New York Eye Cancer Cente

  1. By Henry J. Kaplan, MD. University of Louisville. Co-author (s): Niloofar Piri, MD, University of Louisville. Uploaded on May 2, 2013. Last modified by Chayal Patel on May 7, 2013. Rating 1 rating
  2. Melanocytoma of the ONH is a hamartoma which consists of large round or oval heavily pigmented melanocytes packed closely and located among axons in the optic disc, anterior optic nerve and in the peripapillary retina
  3. Optic nerve melanocytoma is a variant of melanocytic nevus that has typical clinical and histopathologic features. In addition to occurring in the optic nerve, melanocytoma can also be found in the uveal tract, including the iris, ciliary body, and choroid. Most ophthalmologists are not familiar with these extrapapilary melanocytomas
  4. Melanocytoma is a benign intraocular lesion composed of a uniform array of deeply pigmented, plump, polyhedral nevus cells.1It occurs most commonly on or adjacent to the optic disc, although it may appear in the choroid, ciliary body, iris, conjunctiva, or sclera
  5. Uploaded on Mar 13, 2013. Last modified by Caroline Bozell on Mar 14, 2013.; Reviewed by Caroline Bozell; Rating Appears in Miscellaneous Condition/keywords melanocytoma Photographe
  6. Pigmented spindle cells were present on the retina, composing a neoplastic epiretinal membrane, and pigmented vitreous cells were noted (Figure 2E and F). The histopathologic diagnosis was optic nerve melanocytoma with malignant transformation into low-grade melanoma. There was no clinical evidence of metastasis
  7. The differential diagnosis of melanocytoma of the optic disc includes juxtapapillary choroidal melanoma, choroidal nevus, hyperplasia of the RPE, hypertrophy of the RPE, and combined hamartoma of the retina and RPE and adenoma of the RPE. 20 Although extremely rare, malignant melanoma can occur in the optic nerve and may be difficult to.

Optic Nerve Melanoctyoma: a Teaching Case Report The

Histologically, it corresponded to a heavily pigmented choroidal tumor that had broken through Bruch's membrane to infiltrate the overlying retina . Bleached sections showed a uniform population of large cells with abundant cytoplasm and small, bland nuclei consistent with the diagnosis of melanocytoma ( Figure 5 ) The retinal component of the melanocytoma is intensely hypoautofluorescent (white arrow). The subretinal pigments tracking inferiorly from the lesion can also be seen (black arrow); d) SD-OCT image of the cross section at line d in 1c. The raised hyperreflective lesion on the optic nerve head with posterior back shadowing can be seen.

Melanocytoma of the Optic Disk: A Review - ScienceDirec

Melanocytoma is a benign pigmented tumor that may be misdiagnosed as a malignant choroidal melanoma. Melanocytomas may have unusual changes such as spontaneous necrosis of retina and choroid with pigment proliferation. Retinal Cases and Brief Reports1 (1):36-37, Winter 2007. Separate multiple e-mails with a (;) Melanocytoma is a peculiar variant of nevus that classically occurs in the optic nerve head. It may be confined to the optic disc or it may have contiguous involvement of the choroid or sensory retina. It is important to differentiate this benign lesion from a malignant melanoma Optic disk melanocytomas are typically located at the inferior or inferotemporal edge of the optic disk and, in many cases, infiltrate the adjacent choroid and/or the retina. Characteristics features are the dark, almost black color of the tumor and the feathery aspect of its margin in the case of invasion of the nerve fiber layer Iris Melanocytoma: Dark pigmented brown lesion -may have granular mound of black sand appearance.(1) Serous retinal detachment may occur as tumor grows + cause ↓ vision + difficulty visualizing tumor. Exophytic tumors: grow beneath retina → retinal detachment

Optic Disc Melanocytoma - Retina Toda

Melanocytoma of optic nerve (MON) is a rare primarily benign lesion, but due to its malignant potential—estimated around 1-2% of cases (Shields et al. 2006), possible complications and the clinical similarities with other pigmented optic nerve lesions, such as malignant melanomas, one should be able to know and recognize clinical features, hence providing adequate follow-up and assistance TY - JOUR T1 - Vitreous seeding associated with melanocytoma of the optic disc. AU - Lauritzen,K, AU - Augsburger,J J, AU - Timmes,J, PY - 1990/1/1/pubmed PY - 1990/1/1/medline PY - 1990/1/1/entrez SP - 60 EP - 2 JF - Retina (Philadelphia, Pa.) JO - Retina VL - 10 IS - 1 N2 - The authors describe an unusual case of melanocytoma of the optic disc associated with seeding of extracellular pigment. Melanocytoma is a heavily pigmented tumor seen frequently in the optic nerve head but which can arise anywhere in the uvea MELANOCYTOMA. Melanocytoma is an unusual congenital and nonhereditary variant of melanocytic nevus that is classically located on the optic disc. 9 Usually there is a surrounding peripapillary choroidal nevus. Melanocytoma can also be located in the iris, ciliary body, or choroid Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a typically benign, asymptomatic, pigmented fundus lesion. It is a congenital hamartoma of the retinal pigment epithelium (RPE) and occurs in three variant forms: solitary (unifocal), grouped (multifocal) and atypical. Atypical CHRPE is associated with familial adenomatous.

Melanocytoma is a form of nevus that can occur in and behind the iris in the ciliary body. In the image seen below, this melanocytoma has a cobblestone textured surface and feathered margins. Interestingly, it is also causing a small amount of pupillary distortion (correctopia). Bits of iris melanocytoma can break off and settle in the inferior. Melanocytoma of the Optic nerve head, is a benign, stationary pigmented tumor present on or adjacent to the optic disc. Rarely it exhibits minor enlargement and can also undergo malignant transformation to a melanoma. Histopathologically a melanocytoma consists of intensely pigmented round to oval nevus cells with benign features. We report a case of a 77 year old lady with an Optic nerve head. Primary melanocytic tumours of the central nervous system (CNS) form a rare entity which is histologically and clinically distinct from metastatic cutaneous or retinal malignant melanoma. They can be classified into diffuse melanocytosis (diffuse melanosis), malignant melanoma and benign melanocytoma with a small number of intermediate variants

Melanocytoma is a benign melanocytic naevus that can occur in the iris, ciliary body, choroid or optic disc. In the optic disc, this tumour manifests as a darkly pigmented, homogenous mass often with invasive or compressive effects on the adjacent disc structure, as well as the retina, choroid and vitreous. Melanocytoma can simulate melanoma The term melanocytoma was first used in 1962 by Zimmermann to describe a benign, asymptomatic hamartomatous tumor that arises from melanocytes [ 1 ]. It needs to be differentiated from the other lesions of the optic disc in view of the grossly varied treatment and prognosis of these conditions

Central retinal vascular obstruction secondary to

  1. DIAGNOSIS: Melanocytoma of the iris and ciliary body CLINICAL COURSE: Post-biopsy, management options were discussed with the patient and his family. Surgical excisional biopsy was discussed as an option along with the potential complications, such as cataract and retinal detachment. The low risk of future malignant transformation was discussed
  2. melanocytoma: ( mel'ă-nō-sī-tō'mă ), 1. A pigmented tumor of the uveal stroma. 2. Usually benign melanoma of the optic disc, appearing in markedly pigmented people as a small deeply pigmented tumor at the edge of the disc, sometimes extending into the retina and choroid; malignant metaplasia is rare. [megalo- + cyto- + G. -oma; tumor
  3. Discussion. Our patient presented with: a normal optic nerve (except for the incidental finding of melanocytoma OS), normal dilated fundus exam, mild vitreous cells, decreased vision OS associated with RAPD, photopsias, headache, VF loss, and depression of retinal function OS illustrated by the MERG
  4. c. Combined hamartoma of the RPE and retina. d. Melanocytoma. 3. What is the most appropriate treatment? a. Iodine-125 plaque radiotherapy. b. Enucleation. c. External beam radiation followed by enucleation. d. Observation. 4. What is the likely 5-year mortality rate for this patient? a. 0%. b. 15%. c. 40%
  5. Phone: (787) 919-3156. Home Ocular Oncology Retina Pediatrics Physician Resources Contact. Scroll. Ocular. Oncology. OCULAR ONCOLOGY. Villegas MD specializes in diagnosis and treatment of tumors involving the eye. Ocular tumors can affect children and adults. Tumors may occur in one or both eyes
  6. Melanocytoma; Congenital hypertrophy of the retinal pigment epithelium; Management General treatment. Management options include observation, laser photocoagulation, transpupillary thermotherapy, radiotherapy, local resection, or enucleation. Medical therapy. Chemotherapy and immunotherapy are not used for local control of ocular melanoma.

Neuroretinitis associated with melanocytoma of the optic disk. Retina (Philadelphia, Pa.) , 14 (2), 173-6. García-Arumí J, et al. Neuroretinitis Associated With Melanocytoma of the Optic Disk Optic nerve head melanocytoma (ONM) typically appears as black or dark-brown tumors with feathery or fuzzy margins located on the optic disc that often extend into the adjacent retina, choroid and vitreous.1 ONM was first reported in 1907 by Coats, who suspected melanocytomas were benign tumors. In 1962 Zimmerman and Garron described th Although melanocytoma may occur at all sites of the uveal tract, including the iris, ciliary body and choroid,4, 5 optic disc is the most common site. Isolated choroidal melanocytoma not associated with optic disc melanocytoma is rare and difficult to differentiate from melanoma clinically Background. Ocular (oculodermal) melanocytosis is a congenital condition characterised by dermal pigmentation along the first and second divisions of the trigeminal nerve such as the ocular coats and periocular skin.1 It has been well established in literature that ocular melanocytosis predisposes to uveal malignant melanoma.2-11 Melanocytoma is a low-grade pigmented tumour arising from the. Autofluorescence imaging in the differential diagnosis of optic disc melanocytoma Panagiotis Salvanos,1,2 Tor P. Utheim,3,4 Morten C. Moe,1,2 Nils Eide1* and Ragnheiður Bragadόttir1,2* 1Department of Ophthalmology, Oslo University Hospital, Oslo, Norway 2University of Oslo, Oslo, Norway 3Department of Medical Biochemistry, Oslo University Hospital, Oslo, Norwa

Optic Disk Melanocytoma and Optical Coherence Tomography

Traditionally, melanocytoma was considered an indolent lesion that does not grow; however, researchers have noted that 15% of these lesions show some slow enlargement over a number of years. 1 Interestingly, there is a small risk of malignant transformation in an estimated 1% to 2% of cases. 1 Risk factors for malignant transformation include. Differential DiagnosisThe differential diagnosis of melanocytoma of the optic disk includes juxtapapillary choroidal melanoma, choroidal nevus, hyperplasia of the RPE, combined hamartoma of the retina and RPE and adenoma of the RPE, metastatic melanoma to optic disk, epipapillary vitreous hemorrhage, and other conditions. 48,61,62 The detailed. Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas.

Diagnosis: Melanocytoma, Histology: Comment to photo: After depigmentation cells with a large cytoplasm and pale small nuclei become visible. Author(s) Melanocytoma is a benign melanocytic naevus that can occur in the iris, ciliary body, choroid or optic disc. In the optic disc, this tumour manifests as a darkly pigmented, homogenous mass often with invasive or compressive effects on the adjacent disc structure, as well as the retina, choroid and vitreous. Melanocytoma can simulate melanoma. In a 2004 report on 115 patients with optic disc. MELANOCYTOMA Melanocytoma is an unusual congenital and non-hereditary variant of melanocytic nevus that is classically located on the optic disc.9 Usually there is a surround-ing peripapillary choroidal nevus. Melanocytoma can also be located in the iris, ciliary body, or choroid. It is usually composed entirely of maximally pigmented

Abstract. An 11-year-old male, neutered European cat was presented for anisocoria due to pupillary dilation in the right eye. Ophthalmic findings were restricted to this eye and consisted of a raised, darkly pigmented, retrolental mass associated with retinal detachment. Ultrasonography identified the mass lesion protruding into the vitreous. Histopathologic study revealed a juxtapapillary choroidal melanocytoma The pigmented tumor cells stained positive for HMB45 and vimentin but negative for S100, GFAP, NSE, and MNF116. No tumor cells stained positive for the proliferation marker Ki67. These findings conclude that choroidal melanocytoma may mimick choroidal melanoma Purpose . To describe a case of choroidal melanocytoma mimicking a melanoma. Methods . Retrospective case report. Patient . A 48-year-old Moroccan woman presented with progressive, painless decreased vision in her left eye for 2 months. Results . Her visual acuity was light perception in the left eye and 20/20 in the right one

Melanocytoma of the Iris and Ciliary Body

Uveal melanoma is a malignant neoplasm that derives from pigmented melanocytes of the uvea and involves, in order of decreasing prevalence, the choroid, ciliary body and iris. Its prognosis is related to histopathologic and genetic features, tumor size and location, extraocular extension. The diagnosis is fundamentally based on clinical evaluation (ophthalmoscopy, biomicroscopy) and. Diffusely the retina is absent and the remaining underlying retinal pigment epithelium is hypertrophied (tombstoning). MORPHOLOGIC DIAGNOSIS: Eye, uvea: Melanocytoma, German shepherd dog, canine. Signalment (AFIP 2348405): Tissue from a cat . HISTORY: There is a white to pale yellow-colored nodule involving the ciliary body and iris In addition, melanomas undergo rapid growth which accelerates after a period of more quiet progress,1 in contrast to the static nature of the melanocytoma.Hyperplasia of the retinal pigment epithelium is readily seen by slit lamp ophthalmoscopy to occur deep in the retinal structure without invasion of the disc Capillary Hemangioblastoma of the Retina and von Hippel-Lindau Disease. 135. Tuberous Sclerosis and the Eye. 136. Phakomatoses. 137. Retinal Metastases. 138. Remote Effects of Cancer on the Retina. 139. Melanocytoma of the Optic Disc. 140. Congenital Hypertrophy of the Retinal Pigment Epithelium. 141. Combined Hamartoma of the Retinal Pigment. Optic disc melanocytoma (ODMC) was a benign pigmented intraocular tumor with the rare potential malignant transformation. This study was designed to evaluate tumor vasculature with optical coherence tomography angiography (OCTA) in ODMC. Eyes of healthy individuals in a control group and of patients with ODMC were imaged by OCTA systems operating at 840 nm wavelengths and examined

Video: Optic Disc Melanocytoma: Imaging With OCTA - Retina Toda

Figure 1. Melanocytoma of the optic nerve head first detected in a 15-year-old girl. The tumour is sited partly on the nerve head, partly in the uveal tract and shows several pale lacunae and a small degree of feathered edge on the retina where pigment extends along individual nerve fibres. Three years later there was no detectable change in the tumour as evidenced by comparing retinal. 3. Discussion. Optic disc melanocytoma is a benign pigmented tumor arising from melanocytes that rarely affects vision. The most common site of melanocytoma is the optic disc; however it may rarely arise in the orbit. [] In a minority of patients, complications may develop such as vascular occlusion, choroidal neovascularization, polypoidal choroidal vasculopathy (PCV), visual field defects. Posterior Segment Tumors 15.2.1 Retinal Tumors Melanocytoma Melanocytoma is most frequently observed at the optic disc more often in pigmented individuals, although these lesions may sometimes involve the choroid or iris. Unlike in choroidal nevus or choroidal melanoma, the typical optic disc melanocytoma infiltrates the retina, and retinal disorganization may be observed with spectral-domain.

Melanocytoma - Retina Image Ban

Adenoma and adenocarcinoma of the retinal pigment epithelium should also be considered. In this case, FNAB did not establish the diagnosis of a malignant melanocytoma and clearly demonstrates the potential danger of relying on FNAB (alone) for the diagnosis in atypical and necrotic intraocular tumours combined hamartoma of the retina and RPE must first be ruled out (1,3,4). Other differential diagnoses include choroidal nevus, RPE hyperplasia, and RPE adenoma(1,4). Transformation of melanocytoma into malig-nant melanoma is extremely rare(6). OCT is a valuable tool for examining the retina and inner choroid architecture(7). OCT patterns and. Diagnosis of melanocytoma is through direct visualization using slit lamp, gonioscopy, or indirect ophthalmoscopy. Melanocytomas can occur anywhere along the uveal tract, although the optic nerve is the most common site D31.20 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2021 edition of ICD-10-CM D31.20 became effective on October 1, 2020. This is the American ICD-10-CM version of D31.20 - other international versions of ICD-10 D31.20 may differ. All neoplasms are classified in this chapter. Melanocytoma. Benign, deeply pigmented tumor of the optic disc, anterior to the lamina cribrosa; Slightly raised, and typically found eccentrically on the optic disc; Can extend into the retina or posteriorly to the lamina and into the optic nerve; Rarely becomes malignant; Essentially a nevus of the optic dis

PURPOSE: To report findings of optic disc melanocytoma (ODM) obtained using spectral domain optical coherence tomography (SD OCT), with special reference to the central retinal artery and vein surrounded by hyperreflective dots. METHODS: Retrospective review of five eyes of five patients with ODM Ocular melanomas, although rare, are the most common eye tumor in dogs. Ocular melanomas can originate from the uvea or the limbus. About 80% of uveal melanomas (and all limbal melanomas) are benign. The rate of metastasis is less than 5%. Ocular melanomas are at least in part heritable and caused by one or more genetic mutations. Uveal melanomas can become discrete, raised pigmented masses.

Melanocytic Tumors of Iris Stroma | Ento Key

Melanocytoma of Optic Disc: Clinical Manifestations

Melanocytoma of the optic disc was first described by Zimmermann, as a benign hamartoma arising from melanocytes. It is a rare deep-pigmented pathology that affects partial or whole optic nerve with characteristic connection with neighboring retina and choroid. Melanocytoma was perplexed with melanoma of the choroid The Fundus on Phone is extremely compact by design, with the width of the system measuring just 6.8cm and weight over 1.5kg. The design allows it to be used even in the most space-starved settings! It can be used in mass screening programs such as screening for Cataract, Glaucoma and Diabetic Retinopathy. HANDHELD. SLIT LAMP MOUNTABLE Eyes with choroidal and retinal tumors including CN, CM, choroidal freckle, circumscribed choroidal hemangioma (CCH), choroidal metastatic cancer, optic disc melanocytoma (ODM), retinal astrocytic. The latest installment in SLACK's Pocket Guide series, The Pocket Guide to Medical Retina provides essential information on medical retina diseases along with multimodal imaging of each condition, perfect for medical students, residents, fellows, or comprehensive ophthalmologists. This accessible small handbook covers the basics while allowing readers to quickly synthesize information easily.

Melanocytoma (Hyperpigmented Magnocellular Nevus) of The

Introduction. Usually unilateral, posterior melanocytoma presents clinically as an intense black or dark brown mass that may be confined to the optic disk and deeper portions of the optic nerve, but it frequently extends into the nerve fiber layer of the retina and the choroid. 1,2 Although suspected to be a congenital lesion, optic disk melanocytoma is generally observed in adulthood, with a. Benign eye tumour. Ophthalmoscope view of a melanocytoma (dark mass) on the retina (light-sensitive membrane). The melanocytoma is a benign (non-cancerous) tumour, which has grown over part of the optic disc (centre). The optic disc is the region where the optic nerve and retinal blood vessels enter the eye ICD-9-CM 224.5 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 224.5 should only be used for claims with a date of service on or before September 30, 2015. For claims with a date of service on or after October 1, 2015, use an equivalent ICD-10-CM code (or codes). Benign neoplasm, L retina

Bilateral retinal ischemia; Diabetes type 1 with retinal ischemia; Diabetes type 2 with retinal ischemia; Ischemia of retina of bilateral eyes; Ischemia of retina of left eye; Ischemia of retina of right eye; Left retinal ischemia; Retinal ischemia (eye condition); Retinal ischemia due to type 1 diabetes mellitus; Retinal ischemia due to type 2. - retina - meninges. intermediates in melanin formation. start with Tyrosine 1. Dopa (dihydroxyphenylalanine) 2. Dopaquinone 3. Eumelanin or Pheomelanin 4. Melanin melanocytoma - originates from melanocyte - grossly: black mass - benign - dogs: most cutaneous melanocytic tumors are benign Handbook of Pediatric Retinal Disease-Kenneth W. Wright 2007-05-26 Based on Dr. Wright's classic major reference, Handbook of Pediatric Retinal Disease outlines the latest findings in diagnosing retinal disorders in children's and the best available treatment options Rright: Color fundus photograph shows melanocytoma presenting as an elevated brownish-black pigmented lesion, involving most of the optic disc and adjacent retina in left eye; Left: Short-wave autofluorescence image, the melanocytoma reveals hypo-autofluorescence cor-responding to the pigmented masses and adjacent retina. Table 1

The retinal nerve fiber layer was thin adjacent to these lesions, and this corresponded to the opposite hemifield defect on Humphrey Visual Field testing. Three-dimensional imaging of the melanocytoma revealed an irregular elevation in the peripapillary region ment of the neurosensory retina and retinal thickness of 515 micron in this area (Fig. 3B). The patient was diagnosed as a case of melanocytoma of optic disc with classic juxtafoveal CNV in the right eye. The patient underwent one injection of intravitreal bevacizumab 1.25 mg in 0.05 ml. FFA one month after treatment, showed staining and n

Melanocytoma is a deeply pigmented variant of melanocytic nevus. Although most are stable, some can show very slow growth and tumor seeding, similar to melanoma. Figure 2.19. Deeply pigmented presumed iris melanocytoma inferotemporally in the right eye in a young man as seen in 1972 Melanocytoma of the ciliary body misdiagnosed as iridodialysis Moosang Kim, Seung-Jun LeeDepartment of Ophthalmology, School of Medicine, Kangwon National University, Chuncheon, Republic of KoreaAbstract: A 62-year-old female presented to our institution with dimness of vision in her right eye. On examination, her best corrected visual acuity was 20/100 in the right eye Periodic retinal examination through a dilated pupil is the best means of early detection. Cutting out the tumor and leaving the rest of the eye is not routinely advised for this type of cancer. Opening the eye during surgery would allow the tumor cells to float around into the spaces around the eye, which could spread cancer cells to other. Ocular Manifestations. Osteogenesis imperfecta is predominantly known as a bone disease that can also have systemic manifestations. Blue sclera is the most commonly known ocular sign for osteogenesis imperfecta and it is caused by thin scleral collagen allowing the underlying darker choroid vasculature to be seen WHO classification of eye tumors. Andrew Murphy and Dr Francis Deng et al. The World Health Organization (WHO) classification of eye tumors is a widely used pathologic classification system of neoplasms of the orbit. The current revision, part of the 4 th edition of the WHO series, was published in 2018 and is reflected in the article below 1

Choroidal Malignant Melanoma: 58-year-old female withMelanocytoma and Vitreomacular Traction Syndrome OCTChoroidal Rupture - Vitreoretinal Research GroupAcute and Progressive Outer Retinal NecrosisScleral Indentation & Retinal Peripheral Pathology atSarcoidosis - Vitreoretinal Research Group

Shields JA, Shields CL, Ehya H, et al; Total blindness from presumed optic nerve melanocytoma. Am J Ophthalmol. 2005 Jun139(6):1113-4. Salinas-La Rosa CM; Malignant Transformation of Optic Nerve Melanocytoma into Melanoma Associated with Ocular Ischemic Syndrome and Oculocardiac Reflex: Case Report and Review of the Literature. Semin Ophthalmol. Objective Intraocular melanocytoma is a rare naevus variant that can be located at the optic disc or within the uvea, and belongs to the group of non-epithelial-associated melanocytic lesions. We wanted to gain an understanding of the role of GNAQ , GNA11 and BRAF V600E in the pathogenesis of uveal melanocytoma and in cases of transformation to uveal melanoma and also to perform a differential. Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generall Optic disc melanocytoma is typically less than 2 mm raised darkly pigmented mass lesion, which is known to be stationary or show minimal growth [2]. Melanocytoma classically involves the optic disc and in 18% of the patients it remains confined to the optic nerve head, while 77% cases have retina involvement Optic nerve melanocytoma does not usually produce symptoms or grow. If they slowly grow, optic nerve melanocytoma can produce afferent pupillary defects (30%), subretinal fluid (10%), and an enlarged blind spot (75%). On fundoscopic exam, the optic disc may be swollen, atrophic, or even normal. Central retinal vein occlusion may occur