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Year : 2001  |  Volume : 49  |  Issue : 1  |  Page : 51-53

Malignant melanoma of the choroid with vitiligo

MS. O.E.U Institute of Ophthalmology, Kasturba Hospital, Manipal, India

Correspondence Address:
H Vijaya Pai
MS. O.E.U Institute of Ophthalmology, Kasturba Hospital, Manipal, India

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Source of Support: None, Conflict of Interest: None

PMID: 15887717

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How to cite this article:
Pai H V, Satish K R, Maravoor P. Malignant melanoma of the choroid with vitiligo. Indian J Ophthalmol 2001;49:51-3

How to cite this URL:
Pai H V, Satish K R, Maravoor P. Malignant melanoma of the choroid with vitiligo. Indian J Ophthalmol [serial online] 2001 [cited 2023 Dec 2];49:51-3. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2001/49/1/51/22666

Uveal Melanoma is a tumour arising from the melanocytes in the uveal stroma originating in the neutral crest. Vitiligo, on the other hand, is an acquired condition where the melanocytes are unable to form melanin, leading to depigmentation of the skin. The incidence of melanoma is low in Asians; the risk is about one-third of that of Caucasians. Vitiligo has been reported in patients suffering from cutaneous malignant melanoma, and is considered as a sign of good therapeutic response. We report a rare case of choroidal melanoma in an Asian patient who presented with vitiligo.

  Case report Top

A 60-year-old man presented with excruciating pain in the left eye radiating to the forehead, along with redness, swelling and total loss of vision of 6 months' duration. This was preceded by a stick injury to the same eye a week before. The pain subsided after treatment at a local hospital, but the vision did not improve. Pain recurred four days prior to reporting to us. He had noticed white patches over the face and limbs one and half months earlier. There were no other illnesses.

Ocular examination revealed a visual acuity of 6/9 in the right eye and absence of light perception in the left eye. Anterior and posterior segments of the right eye were normal. Hertel exophthalmometry showed a proptosis of 2 mm in the left eye. There was limitation of deorsum duction. A hard mass of 2 x 1.5 cm size was felt through the lower lid. On biomicroscopy, the bulbar conjunctiva showed dilated vessels in the lower part. The cornea showed pigment dusting of the endothelium. Grade II flare was seen in the anterior chamber. The iris showed 360 neovascularisation extending up to the angle, ectropion uvea, and a dilated (6 mm) fixed pupil. Nuclear and posterior subcapsular cataract was present. On fundus examination through the hazy media, a raised solid grayish-black subretinal lesion was seen in the inferior half reaching up to the optic disc and the macula. Due to vitreous haemorrahge, other details of the fundus were unclear. The intraocular pressure was 14 mmHg in right eye and 30 mmHg in the left eye. The patient had an enlarged hard, nontender left preauricular lymph node. Vitiligo patches were seen around the mouth and wrist [Figure - 1].

Computed tomography of the orbit showed a well defined 1 x 1.5 cm mass in the posterior part of the globe with orbital extension [Figure - 2]. A hyperdense lesion with surrounding oedema was seen in the left occipital lobe [Figure - 3]. Goldman visual field of the right eye showed right hemianopia. A thorough systemic examination with radiograph of the chest, liver function tests, and abdominal ultrasonography did not reveal any other evidence of metastasis.

A diagnosis of choroidal melanoma metastatising to the occupational lobe was made. The patient underwent exenteration of the left orbit as palliation. Cut section of the eyeball showed a proliferative white growth involving the posterior and lateral walls of the eyeball, displacing the retina and extending into the surrounding soft tissue [Figure - 4]. On microscopic examination, a mixed cellular pattern with sheets of polygonal cells having large nuclei, prominent nucleoli and scanty cytoplasm, was seen confirming the diagnosis of malignant melanoma. A few of the cells contained melanin [Figure - 5]. Focal areas of tumour necrosis were seen. The optic nerve showed infiltration by tumour cells. A biopsy from the patch of vitiligo was negative for metastasis.

In the postoperative period the patient received chemotherapy at 2-week intervals. The treatment included Vincristine 2 mg intravenously on day one, Lomustin (CCNU) 20 mg orally on day one, Procarbazine 150 mg daily from day one to ten, and Tamoxifen 20 mg twice daily, to be continued. There was no change in the vitiligo patches during the follow up. At the third course of chemotherapy, the patient had developed widespread metastasis to the liver, lungs. He succumbed to the disease three months later.

  Discussion Top

Prior to treatment, metastasis from melanomas of the ciliary body and choroidal melanomas occur in 0.5 - 6.5% of patients.[1-4] Liver is the most common site of metastasis in patients with malignant melanoma. Our patient had CT scan evidence of tumour metastasis to the occipital lobe at the time of presentation, without any evidence of spread to the liver. Histopathology revealed invasion of the optic nerve, which again is rare. Extension of choroidal or ciliary melanomas to the optic nerve occurred in 3.7% of 432 cases reviewed by Shammas and Blodi.[5] The risk factors for optic nerve infiltration have been identified as increased intraocular pressure, large tumour size, diffuse growth pattern, peripapillary location, epitheloid cell type and tumour necrosis.[6] Spencer emphasized that the absence of light perception is distinctly uncommon in typical cases of uveal melanoma, but was characteristically found in his series of melanomas with optic nerve extension.[7] Thus absence of light perception in an eye with a melanoma may suggest the presence of optic nerve invasion by the tumour. The same finding was observed in our patient.

Ocular melanomas are known to give rise to an immune response in the host, which is the basis of immune therapy for treating malignant melanoma. There have been reports of vitiligo in patients suffering from malignant melanoma of the skin who had a favourable outcome.[8] Our patient had patches of vitiligo over the body for one and half months. The cut section of the tumour did not reveal any pigmentation on gross examination. We presume that both the vitiligo and the amelanotic appearance of the tumour could be due to antibody reaction against the melanin-containing cells, incited by the tumour cells.[9] Appearance of vitiligo in patients with malignant melanoma is considered to be good prognostic sign as regards to the life expectancy. According to Barriere et al it did not carry any significance regarding the outcome.[10] In our patient who had vitiligo at the time of presentation, there was no change in the skin lesions after surgery. The patient developed widespread metastasis to liver and lungs within 3 months and succumbed to the same, thus confirming the doubtful prognostic significance of vitiligo in patients with malignant melanoma.

  References Top

Zimmerman LE, McLean IW. Metastatic disease from untreated uveal melanomas. Am J Ophthalmol 1979;88:524-34.  Back to cited text no. 1
Wagoner MD, Albert DM. The incidence of metastases from untreated ciliary body and choroidal melanoma. Arch Ophthalmol 1982;100:939-40.  Back to cited text no. 2
Gragoudas ES, Egan KM, Seddon JM, Glynn RJ, Walsh SM, Munzenrider JE, et al. Survival of patients with metastases from uveal melanoma. Ophthalmology 1991;98:383.  Back to cited text no. 3
Pach JM, Robertson DM. Metastasis from untreated uveal melanoma. Arch Ophthalmol 1986;104:1624-25.  Back to cited text no. 4
Shammas HF, Blodi FC. Peripapillary choroidal melanomas. Arch Ophthalmol 1978;96:440-45.  Back to cited text no. 5
Shields CL, Shields JA, Yanian DL, Augsburger JJ. Intracranial extension of choroidal melanoma via the optic nerve. Br J Ophthalmol 1987;71:172-76.  Back to cited text no. 6
Spencer WH. Optic nerve extension of intraocular neoplasms. Am J Ophthalmol 1975;80:465-71.  Back to cited text no. 7
Bystryn JC, Rigel D, Friedman RJ, Kopf A. Prognostic significance of hypopigmentation in malignant melanoma. Arch Dermatol 1987;23:1053-55.  Back to cited text no. 8
Rahi AHS. Autoimmune response in uveal melanoma. Br J Ophthalmol 1971;55:793-807.  Back to cited text no. 9
Barriere H, Litoux P, Le-Lay M, Bareau B, Stalder JF, Dreno B. Cutaneous achromia and malignant melanoma. Ann Dermatol Venemlogy 1984;111:991-96.  Back to cited text no. 10


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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