|Year : 1968 | Volume
| Issue : 2 | Page : 63-66
Uveal malignant melanoma with leucoderma
MS Nirankari, KK Khanna, RP Mathur
Medical College, Amritsar, India
|Date of Web Publication||22-Dec-2007|
M S Nirankari
Medical College, Amritsar
Source of Support: None, Conflict of Interest: None
|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 May 30];16:63-6. Available from: http://www.ijo.in/text.asp?1968/16/2/63/37494
| Introduction|| |
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  and THEOBALD  hypothesized its Schwann cell origin while others described its origin from melano-blast. Friedenwald (1932) and REESE  , 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  ). 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|| |
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|| |
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,  JAIN, et al  . In histological reports IKUI et al  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  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)  .
The pathogenesis of vitiligo in absence of any such systemic disorder eludes its satisfactory explanation.
| Summary|| |
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|| |
CORDES F. C. AND COOK R.D.: Simultaneous bilateral primary ocular malignant melanoma. Trans. Amer. Ophth. Soc. 47, 80, (1949).
DUKE ELDER W. S.: Text book of Ophthalmology. Vol. III, p. 2362-64 and 2403. Henry Kimpton, London (1940).
ELSCHNIC A.: Arch. F. Ophth. 76, 509. (1910). Quoted by Jain I. S. and Chancier B. in 6.
FRANK E. C.: Died Clinic-N-America. Vol. 549, No. 3, P. 635. (1965).
IKUI II., KIMUR A K.: (1958). Quoted by Jain I. S. and Chancer B. in 6.
JAIN I. S. AND CHANDER B.: Sympathetic Ophthalmia simulating Vogt-Koyanagi Syndrome. Orient . Arch. Ophth. 2, 36-39, (1964).
LEBER F. AND KRAIINSTOVER A.: Arch. F. Ophth. 45, 164 and 2:31, (1898). Quoted by Reese A.B. (1951) in 9.
MASSON P.: The Pigmented Neuromas (in French) Ann. d'anat. path. 3, 417 and 657, (1926). Quoted by Reese A.B. in 9.
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).
THEOBALD, C. D.: Neurogenic origin of Choroidal Sarcoma. Arch. Ophth. 18, 971, (1937).
[Figure - 1]