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Year : 1995  |  Volume : 43  |  Issue : 3  |  Page : 140-141

Malignant melanoma of the choroid in association with oculodermal melanocytosis: A case report

From Medical Research Foundation, Madras, India

Correspondence Address:
Jyotirmay Biswas
Medical Research Foundation, 18 College Road, Madras 600 006
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Source of Support: None, Conflict of Interest: None

PMID: 8822492

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How to cite this article:
Biswas J, Shanmugam MP, Gopal L. Malignant melanoma of the choroid in association with oculodermal melanocytosis: A case report. Indian J Ophthalmol 1995;43:140-1

How to cite this URL:
Biswas J, Shanmugam MP, Gopal L. Malignant melanoma of the choroid in association with oculodermal melanocytosis: A case report. Indian J Ophthalmol [serial online] 1995 [cited 2023 Dec 10];43:140-1. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1995/43/3/140/25290

Oculodermal melanocytosis (ODM) is a congenital melanocytic hyperpigmentation of the face and ocular tissue. The condition was first described by Ota in 1939. The skin in the distribution of the ophthalmic, maxillary and rarely mandibular divisions of the fifth cranial nerve are involved.[1][2][3][4] Ocular pigmentation most frequently involves the choroid and episclera. The other less common sites of pigmentation are iris, anterior chamber angle, conjunctiva, lens and optic disc in descending order.[1],[5][6][7] The pigmentation in ODM occurs from failure of neural crest cells to migrate to the basal layer of the epithelium. The condition is associated with melanotic tumours in the different parts of the body including the uveal melanoma.[7],[8] In two large series of malignant melanoma of the choroid, the incidence of ODM was 1.5%.[7],[8] ODM is most commonly seen in Orientals and Blacks, and is much rarer in Caucasians.[3],[9][10][11] We report here a case of malignant melanoma of the choroid and ciliary body associated with ODM in a patient who presented with healed anterior uveitis and total cataract.

  Case report Top

A 48-year-old female was seen with complaints of loss of vision in her right eye since one year and recurrent attacks of redness since 10 years. Earlier, she was treated with topical, periocular and systemic steroids. The patient's family history as well as her past medical history were unremarkable.

On examination, her visual acuity in the right eye was light perception and in the left eye was 6/6, N6. A brownish pigmented patch was seen on the right lower cheek below the eyelid. Slit-lamp examination of the right eye revealed large dark, bluish-purple pigmentation in the sclera all around the limbus. A sentinel vessel was seen on the nasal side of the conjunctiva. The pupil was irregular due to posterior synechiae. There was total cataract with uveal pigments seen on the anterior surface of the lens [Figure - 1]. There was no keratic precipitates, aqueous flare or cells. The left eye was normal excepting early nuclear sclerosis and posterior subcapsular cataract. The intraocular pressures were within normal limits. The cataract prevented a fundus view in the right eye.

Ultrasonography revealed a dome-shaped mass in the inferonasal quadrant arising from the choroid and anterior part of the ciliary body. Anterior lesional spikes were of high reflectivity and the intralesional spikes were of low to moderate reflectivity. The mass was approximately 12.4 mm in height, while the base of the tumour measured 15 mm vertically and 10.9 mm horizontally.

The investigations done were normal total and differential white blood cell counts; erythrocyte sedimentation rate (ESR) at 40 mm on first hour; positive antinuclear antibody (ANA) - IgM - high positive and IgG - low positive; and negative LE cell phenomenon. Evaluation in a cancer institute revealed metastasis in the liver and an enlarged para-aortic lymph node. A diagnosis of healed anterior uveitis, complicated cataract and malignant melanoma of the ciliary body and choroid was made. The right eye was enucleated. The patient, however, declined chemotherapy for the systemic metastasis.

  Pathology Top

The globe measured 27 mm anterioposteriorly, 23 mm horizontally and 23 mm vertically. Multiple patches of pigmentation were seen around the limbus. Anterior chamber angle was closed on one side. A deeply pigmented tumour mass was found to be arising from the ciliary body and anterior part of the choroid. The iris and ciliary body were deeply pigmented. Pigmentation was also seen within the sclera [Figure - 2]. Anterior portion of the choroid revealed a dark pigmented mass. The overlying retina was detached and atrophic. The bleached section of the tumour showed predominance of spindle A cells and occasional spindle B cells with a few epithelioid cells [Figure - 3]. There was no extrascleral extension. A diagnosis of mixed cell malignant melanoma of the ciliary body and choroid with ocular melanocytosis was made.

  Discussion Top

The first case report of ODM in association with choroidal melanoma was described by Hulke.[12] Since then, several cases have been reported.[1],[9] In our series of 44 cases of malignant melanoma seen in the last 10 years this is the only case (2.7%) associated with ODM.

The presentation of anterior uveitis and complicated cataract in our patient was unusual. Uveitis and complicated cataract have been rarely described in malignant melanoma of the choroid and ciliary body. The uveitis could be due to dispersion of melanin pigments. The fact that the other eye which had no melanosis did not have any episode of uveitis supports the suggestion that the uveitis was probably due to the tumour.

The presence of ocular or oculodermal melanocytosis should warn an ophthalmologist to look for evidence of melanoma of the uveal tract. Such patients should also be advised periodic ophthalmic examination and to report if there are any symptoms such as loss of visual field and blurring of vision, so that diagnosis can be made at an early stage. If pigmented lesion is seen in the fundus, it should be carefully evaluated with ultrasonography. Photographic documentation is useful to assess any increase in size so that prompt and definitive treatment could be instituted.

  References Top

Gonder JR, Shields JA, Albert DM. Malignant melanoma of the choroid associated with oculodermal melanocytosis. Ophthalmology 88:372-376, 1981.  Back to cited text no. 1
Cowan TH, Balistocky M. The nevus of Ota or oculodermal melanocytosis-The ocular changes. Arch Ophthalmol 65:483-492, 1961.  Back to cited text no. 2
Fitzpatrick TB, Zeller R, Kukita A, et al. Ocular and dermal melanocytosis. Arch Ophthalmol 56:830-832, 1956.  Back to cited text no. 3
Ota M, Tanino H. Naevus fusco-caeruleus ophthalmomaxillaris. Tokyo Med J 63:1243-1245, 1939.  Back to cited text no. 4
Teekhasaenee C, Ritch R, Rutnin U, et al. Ocular findings in oculodermal melanocytosis. Arch Ophthalmol 108:1114-1120, 1990.  Back to cited text no. 5
Gonder JR, Nichol J, Augsburger JJ, et al. Ocular and oculodermal melanocytosis. Can J Ophthalmol 20:176-178, 1985.  Back to cited text no. 6
Gonder JR, Shields JA, Albert DM, et al. Uveal malignant melanoma associated with ocular and oculodermal melanocytosis. Ophthalmology 89:953-960, 1982.  Back to cited text no. 7
Velazquez N, Jones IS. Ocular and oculodermal melanocytosis associated with uveal melanoma. Ophthalmology 90:1472-1476, 1983.  Back to cited text no. 8
Dutton JJ, Anderson RL, Schelper RL, et al. Orbital malignant melanoma and oculodermal melanocytosis. Report of two cases and review of the literature. Ophthalmology 91:497-507,1984.  Back to cited text no. 9
Nik NA, Glew WB, Zimmerman LE. Malignant melanoma of the choroid in the nevus of Ota of a black patient. Arch Ophthalmol 100:1641-1643, 1982.  Back to cited text no. 10
Wilkes TD, Uthman EO, Thornton CN, et al. Malignant melanoma of the orbit in a black patient with ocular melanocytosis. Arch Ophthalmol 102:904-906, 1984.  Back to cited text no. 11
Hulke JW. A series of cases of carcinoma of the eyeball. Royal London Ophthalmol Hosp Rep 3:279-286, 1861.  Back to cited text no. 12


  [Figure - 1], [Figure - 2], [Figure - 3]

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