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Pathology of Melanocytoma of Optic Disc

Dr Sampurna Roy MD



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Melanocytoma , a benign melanocytic tumour is a distinctive naevus composed of large polyhedral cells that are packed with melanosomes. 

It is the most common tumour of the optic nerve head. Some cases occur in the ciliary body.

A benign melanocytoma may have some degree of decreased  visual acuity, progressive nerve fiber bundle defect, certain amount of pigment dispersion into the vitreous, slight macular exudation and extension of the tumour tissue into the peripapillary area of the retina.

In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy.

More importantly, it can exhibit malignant transformation into melanoma in 1-2% of cases. 

The melanocytoma is dark brown to black in colour. The mean tumour diameter is 2 mm and the mean thickness is 1 mm.

Associated findings included a choroidal component, retinal component, optic disc edema, retinal edema, localized subretinal fluid, retinal exudation and in rare cases retinal hemorrhage, vitreous seeds and retinal vein obstruction. 

Microscopic features: Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. The cytoplasm of the cells in melanocytoma is packed with melanosomes.

There are two types melanocytes:

(i) Giant melanosomes (ii) Small round melanosomes (characteristic of uveal melanocytes).

Cytologically the lesion is uniform and there is no evidence of pleomorphism. The polyhedral tumour cells may be compressed by nerve fibres and other structures of the optic nerve head.


Further reading

Optic disc melanocytoma and glaucoma.

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

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

Melanocytoma of the optic disk: a review.

Clinical and histopathological studies of melanocytoma of the optic disc .

Melanocytoma: nomenclature, pathogenesis, natural history and treatment.

Clinical and histopathological studies of melanocytoma of the optic disc

Case reports of three patients showing optic nerve head melanocytoma and systemic hypertension.

Melanocytoma inducing compressive optic neuropathy: the ocular morbidity potential of an otherwise invariably benign lesion.

Bilateral optic disk melanocytoma in a 10-month-old infant.

Melanocytoma of optic disc in 115 cases: the 2004 Samuel Johnson Memorial Lecture, part 1.

Diagnostic difficulties in pigmented tumor of the optic nerve head. A case report .



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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)







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