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Intraocular Malignancy - Uveal Melanoma
 
Hemkala L Trivedi*, Vivek Arbhave**, Sujit Murade**, Hemant Todkar**, Rupali Sinha**, Sunil Chawan**
 

A 70 year male patient presented with gradual loss of vision and axial proptosis of right eye over a period of 6 months.

On examination the vision in right eye was found with no perception of light in all the quadrants. On slit lamp examination the right eye showed distorted and oedematous upper lid with chemosis of bulbar conjunctiva. Cornea showed exposure keratitis. Extraocular movements of right eye were absent. On direct and indirect ophthalmoscopy posterior segment details of right eye were not visible. On investigation the lesion was found to be uveal tract malignant melanoma of right eye with extraorbital extension. The patient was advised right eye exenteration with skin graft.

 
Introduction

A choroidol melanoma is a malignant tumour composed of melanocytes pigment containing and producing cells, normally present in the choroid. It is the most common intraocular malignancy in adults. The incidence of melanoma is highest from ages 50-70 and is same in men and women. Very few cases occur in people under 30 years of age. The cause of choroidal melanoma is unknown. Inheritance seems to be of little importance. There is some evidence that environmental factors (carcinogens such as cigarette smoke) may play a role. Choroidal melanomas may produce no symptoms initially if located away from macula.

A tumour that develop next to or in the macula may produce distorted or reduced vision. Tumours located away from the macula may produce changes in the visual field and patient may develop changes in the vitreous and produce flashes of light or floaters. Retinal detachment, glaucoma and rarely pigmentation of sclera are some of the manifestations of the choroidal melanoma.

 
Case Report

A 70 years male patient came in the out patient department of ophthalmology in Nair hospital with gradual loss of vision and axial proptosis of right eye over a period of 6 months (Fig. 1). Patient had history of cigarette smoking for 50 years. On examination the vision in right eye was found with no perception of light in all the quadrants. Extraocular movements of right eye were absent. Right eye upper lid was oedematous and distorted with bulbar conjunctiva showing chemosis . Cornea of right eye showed features of exposure keratitis. Exophthalmometry with Hertels exophthalmometry showed axial proptosis of 36 mm and left eye 20 mm from lateral orbital margin. The right eye showed black mass involving entire eye behind the equator. On direct and indirect ophthalmoscopy the posterior segment details were not visible. The examination of left eye revealed that it was absolutely normal.

On USG-B-scan of right eye showed a dome shaped mass involving uveal tract and the entire posterior segment along with the optic nerve infiltration. The mass had reflectivity and cascading effect and measured about 10.6 X 8.6 mm in size. It also showed inferior exudative retinal detachment of right eye.


Fig. 1 : Axial proptosis Rt eye.

On CT scan with orbital and brain cuts of right eye showed dome shaped mass involving entire eye upto equator, erosions of medial wall and floor of the orbit (Fig. 2).

The lateral wall of right eye showed black pigmentation. Infiltration of the extraocular muscle was also evident. Infiltration of the optic nerve except posterior part of optic nerve near the canal was evident.

Blood investigations, USG abdomen, X-ray chest PA view were normal. The Exenterated mass measured about 8 mm X 10 mm (Fig. 3).

The exenterated mass was sent for histological examination which showed large oval round epithelial cells with well-demarcated cell membranes, eosinophilic cytoplasm and round nuclei with prominent nucleoli. The patient was followed up for about 8 months after surgery and was found to have no metastatic involvement of other organs. Patient later didn’t followed up. The relative of the patient reported after about one year that the patient died due to Juandice in village.

 
Discussion

Choroidol melanoma constitutes 80% of all the intraocular tumours. The incidence is highest in the age group of 50-70 age. The sex ratio is equal for both men and women. Very few cases are recorded below 30 years of age. The cause of choroidal melanoma is unknown. Inheritance seems to be of very little importance. Environmental factors such as cigarette smoke may play a role by influencing benign pigmented cells to change into malignant cells. The white population are more affected than black. Choroidal melanomas are very uncommon in Indian population. Diagnosis can be made clinically, aided by radiological and blood investigations. The blood investigation included liver function test which can reveal metastatic changes in liver. In radiological methods. B-scan, aided with A-mode can reveal the size, shape excavation and shadowing in the orbit. CT-scan and MRI are used to rule out extraocular metastasis.


Fig. 3 : The operated mass measured 8 mm x 10 mm.

Fluorescence angiography is used to detect extensive leakage with progressive fluorescence, late staining of the lesion. X-ray chest and mammography are also used to detect metastasis. Indocyanine green angiography, colour-coded doppler and fine needle aspiration biopsy are also used in diagnosis. Positron emission tomography, 31P magnetic resonance spectroscopy and monoclonal antibody tagged with a short lived radioactive technetium 99m tracer are new modalities of investigation. Mortality of the patients depends upon the types of cells the tumour has.

 
Modalities of Treatment Enucleation
he patient having large tumours more than 100 mm2 and when the vision is irreversibly lost, the choice of treatment is enucleation. Enucleation is performed with gentle isolation and section of the extraocular muscles and minimal traction on the optic nerve when cutting.8 After 7 weeks patient can be fitted with an artificial eye.
 
Trans-scleral Local Resection
Patients having tumours less than 16 mm in diameter are usually selected for trans-scleral local resection. The procedure involves excision of the tumours with a rim of healthy choroid under a partial thickness scleral flap.11
 
Plaque Radiotherapy

Tumours less than 10 mm in elevation and less than 20 mm in basal diameters are treated with plaque radiotherapy. The procedure involves outlining the tumour with methylene blue which is further outlined with diathermy. A transparent perspex dummy applicator is temporarily centred over the tumour and pre-placed sutures are inserted.

The dummy applicator is replaced with a radioactive plaque of same size. 25I is considered ideal for intraocular tumours. With energy of 0.039 Mev and it lacks alpha or beta rays.12 Other isotopes used are radon with energy range of 0.22 to 2.20 Mev and short life of 3.8 days. And 60Co with energy of 1.77 to 1.33 Mev.6 The disadvantage of these isotopes is high tissue penetration which may cause damage to normal tissue. The response of the therapy can best evaluated by clinical examination, colour photograph and utlrasonography of the tumour. Tumour regression starts after 1-2 months of the treatment.

 
Charged Particle Irradiation
Tumour of 24 mm in diameter and 14 mm in height and also posterior location to within 4 mm of disc or fovea are treated by this therapy. In this therapy four tantalum rings 2.5 mm in diameter are sutured to the sclera to outline tumour.7 Tumours in contact with optic nerve have rings placed at anterior and lateral margins of tumours. After estimating the size, shape, location of tumour, protons or helium are delivered to the eye by means of a cyclotron unit over 4 days with each dose delivered over a 30 sec period. Majority of patients receives a dose of 70 cobalt Gy.1 Tumour regression starts after 6 months of treatment.
 
Transpupillary Thermotherapy
Small tumours located near the fovea and optic disc are treated with this therapy. It can also be a complement modality to brachytherapy. A diode laser is applied for 1 minute to induce hyperthermia.
 
Laser Photocoagulation
Adjunctive therapy for small recurrences after plaque therapy but has been largely suppressed by transpupillary thermotherapy.10
 
Exenteration
Indicated for patients with extensive extraocular extension at the time of diagnosis or for orbital recurrence following enucleation.
 
Palliation
Chemotherapy and immunotherapy may prolong life in patients with metastatic disease. In patients with lung metastasis, life expectancy is generally < 1 year and when liver is involved < 3 months.
 
Conclusion
Choroidal tumour form about 80% of all the intraocular tumours. Incidence is highest between 50-70 age group. Depending upon the size, location, metastasis the mode of management is decided. Initially enucleation was considered as an only treatment. But addition of new modulations like plaque radiotherapy, charged particle irradiation, transpupillary thermotherapy, laser photocoagulation the rate of mortality among the patients has decreased.
 
References
1. Char DH, Saunders W, Castro JR, et al. Helium in therapy for choroidol melanoma ophthalmology 1983; 90 : 1219-25.
2. Char DH, Schwartz A, Miller TR, Abele JS. Ocular metastases from systemic melanoma. Am J Ophthalmol 1980; 90 : 702-07.
3. Char DH, Stune RD, Irvine AR, et al. Diagnostic modalities in choroidal melanoma. Am J Ophthalmol 1980; 89 : 223-30.
4. Constable IJ, Kochler AM. Experimental ocular irradiation with accelerated proton invest. Ophthalmol 1974; 13 : 280-87.
5. Eurle J, Kline RW, Robertson PM. Selection of iodine 125 for the collaborative ocular melanoma study. Arch Ophthalmol 1987; 105 : 763-64.
6. Finger PT. Microwave Plaque thermoradiotherapy for choroidal melanomas. Br J Ophthalmol 1992; 76 : 358-64.
7. Gragoudas ES, Seldon JM, Egan KM, et al. Metastasis from uveal melanoma after proton bean irradiation. Ophthalmology 1988; 95 : 992-99.
8. Jakobiee FA. A meratorium on enucleation for choroidal melanoma. Am J Ophthalmol 1979; 87 : 842-46.
9. Margo CE, McLean IW. Malignant melanomas of the choroid and ciliary body in black patients. Arch Ophthalmol 1984; 102 : 77-79.
10. Meyer Schuviokerath G. Vogel M. Treatment of malignant melanomas of the choroid by photocoagulation. Trans Ophthalmol Sol UK 1977; 97 : 416-20.
11. Meyer Schwickerath G. Excision of malignant melanoma of the choroid. Mod Probl Ophthalmol 1974; 12 : 562-6.
12. Pocker S, Retman M, Salanitro P. Iodine-125 irradiation of choroidal melanoma : Clinical experience. Ophthalmology 1984; 91 : 1700-7.
13. Pockers S, Stollers S, Lesser ML, et al. Long term results of iodine 125 irradiation of uveal melanoma. Ophthalmology 1992; 99 : 767-74.
14. Shields JA. Current approaches to the diagnosis and management of choroidal melanomas. Surv Ophthalmol 1977; 21 : 443-63.
15. Shields JA. Diagnosis and management of intraocular tumours St. Louis, CV Mosby, 1983 pp 75-254.
   

DEMEDICALISING NECK PAIN HELPS PATIENTS

Brief physiotherapy interventions focused on demedicalising the problem and teaching the principles of cognitive behaviour therapy can be as beneficial as standard physiotherapy for patients who prefer this treatment. Klaber Moffett and colleagues randomised 268 patients to standard physiotherapy (five sessions) or up to three sessions of the brief intervention. Overall, patients who received standard care reported a significant improvement compared with patients in the brief intervention group, but patients who preferred the brief intervention and received this treatment had similar outcomes to patients receiving usual physiotherapy.

BMJ, 2005; 330 : 75.

ST JOHN’S WORT IS EFFECTIVE FOR MODERATE TO SEVERE DEPRESSION

Hypericum extracts WS 5570 (St John’s wort) is at least as effective as paroxetine and is better tolerated as a treatment for moderate to severe depression. In a double blind, double dummy, reference controlled, multicentre non-inferiority trials, Szegedi and colleagues randomised 251 adults to 900 mg/day of hypericum extract WS 5570 or to 20 mg/day paroxetine. The decrease in depression score 42 days later was more than 55% for patients taking St John’s wort and less than 45% in the group taking paroxetine.

BMJ, 2005; 330 : 503.


*Associate Professor; **Post Graduate Resident,
Department of Ophthalmology, TNMC and BYL Nair Hospital, Mumbai 400 008.