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Year : 1968  |  Volume : 16  |  Issue : 2  |  Page : 63-66

Uveal malignant melanoma with leucoderma

Medical College, Amritsar, India

Date of Web Publication22-Dec-2007

Correspondence Address:
M S Nirankari
Medical College, Amritsar
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Nirankari M S, Khanna K K, Mathur R P. Uveal malignant melanoma with leucoderma. Indian J Ophthalmol 1968;16:63-6

How to cite this URL:
Nirankari M S, Khanna K K, Mathur R P. Uveal malignant melanoma with leucoderma. Indian J Ophthalmol [serial online] 1968 [cited 2020 Aug 15];16:63-6. Available from: http://www.ijo.in/text.asp?1968/16/2/63/37494

  Introduction Top

Malignant melanoma of the uveal tract is the most common pigmented intra-ocular growth occurring mostly in sixth and seventh decades. Males and females are about equally aff­ected. The lesion in the uveal tract is invariably unilateral but multi focal in origin. There is little evi­dence of hereditary influence al­though transmission through four generations has been recorded.

Malignant melanoma most com­monly forms a circumscribed tumour but occasionally diffusely involves the uvea over a wide area. Much controversy surrounds the mystery of its origin. MASSON [8] and THEO­BALD [10] hypothesized its Schwann cell origin while others described its origin from melano-blast. Frieden­wald (1932) and REESE [9] , accord­ing to their clinico-pathological. studies, described that Schwann melanomas are twice as common as those derived from melanoblasts of the uveal stroma. The present trend is to classify melanomas into benign or malignant without going into their subdivision described above. This is due to the fact that malignant mela­noma of the uvea may arise from pre­existing benign melanoma sponta­neously or due to irritation from in­flammation or perhaps trauma. The classification based on its histological description of cells is much helpful in determining the prognostic value of the growth.

The tumour is rarely diagnosed in its early stages when it may produce some visual disturbances and is invi­sible on ophthalmoscopic examination. In most of the cases, the pa­tient presents with varying compli­cations resulting from the proteolytic products of the wide spread necrosis in the melanoma. These include uveitis, hypopyon, new vessel forma­tion on the iris, secondary cataract, secondary glaucoma, pan-ophthalmitis, orbital cellulitis, vitreous haemor­rhage and retinal detachment.

Most of these secondary changes are due to liberation of toxins from necrosis of the tumours, improper metabolism of the abnormal tumour cells, faulty circulation and chemical changes in the stagnant fluid under the separated retina. The necrosis is thought to be the result of the tumour outgrowing its blood supply. Toxins from the necrosed tissue may even dissolve sclera and the tumour tissue may extend directly through the scleral wall and disseminate itself throughout the orbital tissue besides causing the complications. In the complication of severe inflammation the eye ball may end up with phthisis bulbi and spontaneous cures are even reported but in such circumstances usually some viable portion may re­main in or outside the eye which may resume its growth as late as twenty years (LEBER and KRAHN­STOVER [7] ). Sympathetic ophthal­mitis due to choroidal melanoma has also been reported. Clinically pig­ment content of the nevus has been seen to decrease and infact at times, entire nevus may disappear sponta­neously and completely. Also acquir­ed spontaneous precancerous mela­nosis may appear, regress and reap­pear. Depigmentation (leucoderma) of the skin in association with malignant melanoma has not been found described in the literature. Recently we met with an unusual case of mal­ignant melanoma with leucoderma.

  Case report Top

Chet Ram Singh, 57 years old male, was admitted in Ram Lal Eye Hospital, Amritsar with complaint of a painful and inflamed growth in his right eye socket. The patient nar­rated the following interesting his­tory. Eleven years back he deve­loped severe pain, redness and dimi­nution of vision of his right eye. He was admitted as a case of acute con­gestive glaucoma and a filtering ope­ration was done to relieve it. The patient left the hospital after about a month without much benefit in the condition of his eye. He consulted various practitioners and continued their treatment for about two years when he developed pan-ophthalmitis for which he was re-admitted and the offending eye eviscerated. Fol­lowing this for nine years all went well except for the occasional pain in the right socket. Two years back, he developed patches of leu­coderma on his face, localized mostly on lids, forehead and a few patches on both his legs. In the course of time these patches gradually in­creased in their size. Also about six months back, he noticed a small swelling in his right socket for which he paid little attention as it was not painful. Two months back he again developed severe pain in his right eye socket and right side of the face when the size of the swelling of the right socket increased. This com­pelled him another visit to the hos­pital. When examined, he was well built and nourished. No abnormality was detected in his body systems except patches of leucoderma on his face [Figure - 1] and both his legs with a diffuse growth in his right eye soc­ket. The growth was of the size of a cherry and diffusely spread both inside and outside the scleral wall. It was hard painful vascularized, pig­mented, non-capsulated and adherent with the surrounding structures. The lymph glands in the head and neck were not enlarged. Orbital exente­ration operation was performed and the histopathological report of the biopsy from the growth revealed malignant melanoma.

  Discussion Top

As already stated, the presence of malignant melanoma, especially in its early stages, may be marked by secondary changes in the eye due to necrosis of the tumour. The above patient was initially treated for acute congestive glaucoma which has been a complication of the malignant melanoma present in his eye and which escaped detection. The tumour might have been located in the periphery of the retina in the region of a vortex vein where by its interference with the return of the venous blood, it prompted stasis and caused glaucoma. In other cases glaucoma may be caused by haemor­rhage in the tumour and vitreous or the inflammation set up by the toxins of the necrotic tissue. In the course of time pan-ophthalmitis as a com­plication of the melanoma might have ensued in this patient and the eye had to be eviscerated. There was an apparent cure for about nine years but some viable portion of the extra ocular extension resumed the growth. The baffling problem was the development of vitiligo on face and legs.

Two possibilities could be enter­tained in this regard.

First the acquired leucoderma was just a coincidence and had nothing to do with the progress of the mela­notic tumour of the uvea. But the development of the vitiligo and re­sumption of growth in the eye and their progress simultaneously means something more than a mere coinci­dence. Various forms of hyper-pig­mentation have been observed to be associated with many forms of inter­nal. malignancy and melanoma in particular. The hyper-pigmentation may be due to involvement of adre­nal glands; direct infiltration of the skin by melanotic tumour, prolonged dermatitis and excoriation due to malignancy and in many cases with­out any cause. Hypo-pigmentation of the skin due to malignancy has not been observed except in connec­tion with prolonged dermatitis in dark skinned individuals with lym­phoblastoma.

It will not be a far fetched idea to link the development of leucoder­ma with the inflammation set up by necrosis of the tumour in the uvea.

Sympathetic ophthalmia and Vogt Koyanagi syndrome have been re­ported to develop in patients having malignant melanoma of the uvea (DUKE ELDER, [2] JAIN, et al [6] . In histological reports IKUI et al [5] has reported that epithelial cells of sym­pathetic ophthalmia and Vogt-Koya­nagi syndrome are derived from pro­liferation of pigment cells of uvea. Ikui postulated that in - malignant melanoma, proliferation and disinte­geration of pigment cells may be due to some chromatotrophic virus which may be identical with that responsi­ble for Vogt-Koyanagi syndrome and sympathetic ophthalmia. Alternative­ly destruction of pigment leads to pigment allergy in the body produc­ing vitiligo, graying of hairs etc. ELSCHNIG [3] also explained viti­ligo in Vogt Koyanagi syndrome due to pigment anaphylaxis. We agree with these authors in hypothesizing pigment anaphylaxis responsible for depigmentation. We also feel that pigmentary changes secondary to uveal malignant melanoma may be due to some abnormal neurogenic impulses set up by the malignancy and necrosis of the tissues in the eye. Such neurogenic impulses have been blamed for pigmentary changes in the iris in neurogenic heterochromia and neurogenic essential atrophy of the iris (DUKE ELDER) [2] .

The pathogenesis of vitiligo in ab­sence of any such systemic disorder eludes its satisfactory explanation.

  Summary Top

1. An interesting case of malig­nant melanoma with its complica­tions and development of vitiligo has been presented.

2. It should be borne in mind that the presence of malignant melanoma can be masked by the complications arising out of the necrosis of the tumour. In such cases thorough in­vestigations should be made to ex­clude the presence of the tumour be­fore treating the case as glaucoma, cataract, retinal detachment, orbital cellulitis and inflammation in the eye ball at this age.

3. Vitiligo can be explained only by pigment anaphylaxis or abnormal neurogenic impulses in such a case.

  References Top

CORDES F. C. AND COOK R.D.: Simultaneous bilateral primary ocular malignant melanoma. Trans. Amer. Ophth. Soc. 47, 80, (1949).  Back to cited text no. 1
DUKE ELDER W. S.: Text book of Ophthalmology. Vol. III, p. 2362-64 and 2403. Henry Kimpton, London (1940).  Back to cited text no. 2
ELSCHNIC A.: Arch. F. Ophth. 76, 509. (1910). Quoted by Jain I. S. and Chancier B. in 6.  Back to cited text no. 3
FRANK E. C.: Died Clinic-N-America. Vol. 549, No. 3, P. 635. (1965).  Back to cited text no. 4
IKUI II., KIMUR A K.: (1958). Quoted by Jain I. S. and Chancer B. in 6.  Back to cited text no. 5
JAIN I. S. AND CHANDER B.: Sym­pathetic Ophthalmia simulating Vogt­-Koyanagi Syndrome. Orient . Arch. Ophth. 2, 36-39, (1964).  Back to cited text no. 6
LEBER F. AND KRAIINSTOVER A.: Arch. F. Ophth. 45, 164 and 2:31, (1898). Quoted by Reese A.B. (1951) in 9.  Back to cited text no. 7
MASSON P.: The Pigmented Neuro­mas (in French) Ann. d'anat. path. 3, 417 and 657, (1926). Quoted by Reese A.B. in 9.  Back to cited text no. 8
REESE A. B.: Pigmented tumors. Am. J. Ophth. 27, 217, (1944). Tumors of the eye. p. 235-247. Paul B. Hoeber, Inc. N. York (1951).  Back to cited text no. 9
THEOBALD, C. D.: Neurogenic ori­gin of Choroidal Sarcoma. Arch. Ophth. 18, 971, (1937).  Back to cited text no. 10


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