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| CASE REPORT |
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| Year : 1995 | Volume
: 43
| Issue : 4 | Page : 198-200 |
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Progressive growth in melanocytoma of the optic nerve head
Vishali Gupta1, Amod Gupta1, MR Dogra1, SS Pandav2
1 Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, ChandigarhDepartment of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Correspondence Address:
Amod Gupta
Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh 160 012
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 8655201 
How to cite this article:
Gupta V, Gupta A, Dogra M R, Pandav S S. Progressive growth in melanocytoma of the optic nerve head. Indian J Ophthalmol 1995;43:198-200 |
Melanocytoma of the optic disc (magnocellular nevus), is a benign, pigmented, intraocular tumour that has typical clinical and pathologic features.[1][2][3][4][5] Clinically, this is characterized by a dark brown to black lesion involving the optic disc and histologically is composed of deeply pigmented round to oval cells with abundant cytoplasm and small, round, bland nuclei. Tendency for growth and malignant transformation are not features associated with melanocytoma. However, one large series[5] has documented the subtle enlargement of these tumours in 15% of cases over several years. The enlargement is only minimal and the pattern of growth in these occasionally expanding melanocytomas is not known.[6] The increase in the size of the tumour generally takes several years, although in one case the tumour was shown to grow in a period of one week.[1] The enlargement is essentially benign and, so far, no documented case of malignant transformation has been reported. We report one such rare example of a clinically typical melanocytoma that grew progressively over a period of 6 years.
| Case report | |  |
In 1989, a 32-year-old female was found to have a pigmented lesion in the fundus of the right eye, covering the nasal half of the optic disc [Figure - 1]. The best-corrected visual acuity was 6/6 in both the eyes with normal visual fields. Fundus fluorescein angiography showed hypofluorescence of the mass in both early and late phases [Figure - 2]. Based on its clinical and angiographic appearance, the lesion was diagnosed as melanocytoma of the optic nerve head and the patient was advised to have periodic examinations.
In March 1995, she complained of slight blurring of vision in her right eye. Her best-corrected visual acuity was 6/6 in both eyes and intraocular pressure was 16 mm Hg in each eye. Slit-lamp biomicroscopy of the right eye did not show any pigment clumps in the anterior vitreous cavity. Visual fields of the right eye revealed lower nasal quadrant scotoma. Fundus examination of the right eye disclosed a dome-shaped black mass that obscured the entire optic disc and also the juxtapapillary choroid nasally [Figure - 3]. There was absence of pigment clumps into adjacent retina or vitreous, nerve fibre layer haemorrhage, subretinal exudation, secondary retinal detachment, orange pigmentation or any abnormal vascularity which could otherwise demonstrate the malignant transformation of the lesion. Fluorescein angiography showed a hypofluorescent mass in both early and late phases with absence of leakage around the mass and an obvious increase in the size of the lesion compared to previous angiograms [Figure - 4]. B-scan ultrasonography demonstrated a mass arising from the optic nerve head and juxtapapillary choroid. The mass had acoustic solidity without any choroidal excavation. A-scan ultrasonography showed the mass to have high internal reflectivity [Figure - 5]. CT scan revealed that the mass was not involving the optic nerve. Based on all these findings, the clinical diagnosis remained as melanocytoma of the optic disc with progressive enlargement.
| Discussion | | |
Several features in this case are typical of an optic nerve melanocytoma which demonstrate against its conversion into malignant melanoma. These include no change in the jet black colouration of the mass, the origin from the optic disc as noted on initial presentation, the relatively well-preserved visual acuity and fields over a period of several years, its slow growth pattern, absence of any pre-retinal pigment clumps or plaques and retinal detachment. On ultrasonography, the lesion showed acoustic solidity, no choroidal excavation and high internal reflectivity; features not suggestive of malignant melanoma. Further, fluorescein angiography did not show any leakage or staining in the mass lesion.
Malignant transformation of extrapapillary uveal melanocytic nevus has been recognised[1] but the malignant transformation of a true melanocytoma affecting the optic disc has rarely, if ever, been recorded. Zimmerman[7] presented a case diagnosed initially as a low-grade melanoma arising from the melanocytoma of the optic disc which later, however, was reported as documented growth of melanocytoma without malignant transformation.[6] Apple et al[8] described the first case of malignant transformation of an optic nerve melanocytoma. However, the diagnosis remained doubtful as the initial lesion was not compatible with a melanocytoma of the optic disc. Shields et al[9] have also reported a case of malignant transformation of melanocytoma of the optic disc and juxtapapillary choroid. However, the authors could not conclude whether the transformation occurred in the juxtapapillary choroid and invaded the optic nerve secondarily or whether it occurred in the optic disc melanocytoma. Thus, as of now, there is no proven case of malignant transformation of melanocytoma of the optic disc and the enlargement of the disc lesion in our case most probably represents a benign enlargement of the melanocytoma.
| References | | |
| 1. | Zimmerman LE. Melanocytes, melanocytic nevi, and melanocytomas. The Jonas S. Friedenwald Memorial Lecture. Invest Ophthalmol Vis Sci 4:11-41, 1965.  |
| 2. | Zimmerman LE, Gannon LK. Melanocytoma of the optic disc. Int Ophthalmol Clin 2:431-440, 1962. |
| 3. | Shields JA. Diagnosis and management of intraocular tumors. St. Louis, CV Mosby, 1983, pp. 595-616. |
| 4. | Shields JA. Melanocytoma of optic nerve head: A review. Int Ophthalmol 1:31-37,1978. |
| 5. | Joffe L, Shields JA, Osher RH, Gass JDM. Clinical and follow-up studies of melanocytomas of the optic disc. Ophthalmology 86:1067-1078,1979. |
| 6. | Mansour AM, Zimmerman L, La Piana FG, Beauchamp GR. Clinicopathological findings in a growing optic nerve melanocytoma. Br J Ophthalmol 73:410-415, 1989. |
| 7. | Zimmerman LE, Spindle A. Melanoma emerging from a melanocytoma of the optic disc. Presented at Verhoeff Society Meeting, June 1975, Washington DC. |
| 8. | Apple DJ, Craythorn JM, Reidy JJ, Steinmetz RL, et al. Malignant transformation of an optic nerve melanocytoma. Can J Ophthalmol 19:320-325, 1994. |
| 9. | Shields JA, Shields CL, Eagle RC, Lieb WE, et al. Malignant melanoma associated with melanocytoma of the optic disc. Ophthalmology 97:225-230, 1990. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
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