Retinoblastoma - demographic features, clinical presentations, treatment modalities and outcomes in a tertiary care centre

Preethi, S (2006) Retinoblastoma - demographic features, clinical presentations, treatment modalities and outcomes in a tertiary care centre. Masters thesis, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai.


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INTRODUCTION: Retinoblastoma is the most common primary intraocular malignancy of infancy and childhood1. It is the second most common intraocular malignant tumour of all age groups next to choroidal melanoma. It is the tenth most common cancer in childhood in US and accounts for 3% of all childhood cancers2. Retinoblastoma has cumulative life time incidence of 1:18000 to 30,000 live births world wide3. Indian studies have shown the incidence of the tumour in India as 1:15000 live births (Viswananth et al). The Aravind Eye Care System is one of the few tertiary eye care centers in India that has all facilities for diagnosis and treatment of Retinoblastoma and the hospital is also involved in research on its various aspects. The hospital acts is one of the main referral hospital in south India. A prospective study conducted in cases of Retinoblastoma treated in this hospital would give a good idea about demography, main mode of presentation, clinical features, acceptance and response to treatment of the cases in this part of the country and would help us in proposing new strategies or strengthening existing ones for earlier diagnosis and opting better treatment methods in treatment of Retinoblastoma. AIM: To study the demographic features, clinical presentations, treatment modalities and outcomes in patients with retinoblastoma in a tertiary care centre. OBJECTIVES: 1. To study the demographic features of patients with retinoblastoma. 2. To study the various modes of presentation of patients with retinoblastoma. 3. To study the tumour characters in eyes with retinoblastoma. 4. To study the different treatment modalities for retinoblastoma (including local ophthalmic therapy, chemotherapy, surgery) and outcomes of eyes with retinoblastoma, following treatment. MATERIALS AND METHODS: A Prospective and Descriptive study was carried out in newly diagnosed patients with intraocular retinoblastoma who were identified at the Aravind Eye Hospital, Madurai. India. Patients eligible for consideration were all newly diagnosed cases of unilateral, bilateral or trilateral retinoblastoma who presented between January 2004 and May 2005. All other cases of retinoblastoma previously treated elsewhere, or review patients who were subsequently subjected to chemotherapy during the follow up were excluded from the study. The patients who presented for the first time were examined in the “Retinoblastoma Clinic” and were subjected to a detailed medical and ophthalmologic history taking including age, complaints at presentation, duration of symptom, antenatal, natal, postnatal, diet, immunization & family history including pedigree charting. Assessment of visual acuity was done whenever possible either by ability to pick up cake decoration, ability to fix & follow light or visual acuity by SG chart (Scheridan Gardner Chart) in pre school and school going children. This was followed by diffuse torch light examination, slit lamp examination whenever possible of anterior segment to look for hyphaema, hypopyon, pupillary reaction, secondary glaucoma and other signs of anterior segment involvement. Indirect ophthalmoscopy using 20D lens of the dilated fundus was done to diagnose tumour mass, retinal detachment, vitreous hemorrhage, vitreous seeding, signs of optic nerve head involvement. The diagnosis was confirmed using Ultrasound B Scan. In patients whom diagnosis could not be ascertained or to look for optic nerve infiltration & intracranial extent, a CT Scan Brain and Orbit (Plain and Contrast) was performed. In most of the patients, since they were children and could not cooperate for detailed examination of anterior and posterior segment in the outpatient department, a staging examination under anesthesia was performed and all anterior and posterior segment finding and character of the tumour were confirmed and noted. The patients eyes were staged according to the “Newly proposed classification for Intraocular Retinoblastoma”. General physical examination was done in all patients to assess general health detect a signs of metastatic disease. Good general and genetic counseling was given for the family regarding the disease and the stage of the disease in their child, need for treatment, regular follow up in the treatment. Any interventional procedure was performed after a written informed consent from parent or guardian. According to the staging of the eye with retinoblastoma, the eye was subjected to either or combination of local therapy, chemotherapy, radiotherapy, surgery. According to the location and size of the tumour Local therapy given was either or combination of cryotherapy, transpupillary thermotherapy, and laser barrage around the tumour. DISCUSSION: In our study, the average age at presentation was 27.54 months (Unilateral cases – 28 months, bilateral cases 22.31 months). The median age at presentation was 24 months (24 months in unilateral cases and 21 months in bilateral cases). The minimum age at presentation was thirteen days and maximum age was 8 years. 89.58% were diagnosed below 5 years of age and 27.08% before age of 1 year 58.33% had unilateral diseases & 41.67% had bilateral disease, 10%of the bilateral cases had bilateral disease. Male female ratio was 1.18:1. CONCLUSION: 1. Our study showed presenting age to be slightly higher than in western literature(The mean age at presentation was 27.54 months (Unilateral cases – 28 months, Bilateral cases 22.31 months). 2. In our study the number of cases that presented to us before the age of 1 year was lesser when compared to the western literature. (27.08% diagnosed before age of 1 year). 3. Male Female Ratio (1.18:1), Percentage of unilateral (58.33%) and bilateral retinoblastoma (41.67%), in our study was similar to previous reports from western world. 4. Percentage of trilateral retinoblastoma (5%) among the bilateral ones in our study was similar to previous reports. 5. Leucokoria(66.66%) was the commonest mode of presentation in our study similar to other studies. But mode of presentation with defective vision(22.91%), glaucoma(22.91%), proptosis(6.25%) were higher in our study compared to western literature. Presentation as strabismus (12.50%) was much lesser in our study. 6. Percentage of cases (86.76%) presenting in advanced stage of disease were higher. 7. The response to attempt to salvage eyes using chemoreduction & local therapy with three cycle VEC regimen seems promising (regression of tumour in 73.33%). Even group D tumours have shown regression completely after chemoreduction, local therapy and EBRT (EBRT was required is one out of 5 Group D cases which showed regression of tumour). 8. Retinal detachment, vitreous seedings and vitreous hemorrhage at presentation could be taken as indicators for poor prognosis for tumour regression. 9. A long term follow up is necessary to assess the long term survival of these patients, and also to diagnose the recurrence of tumour if any in these patients and to decide if more number of cycles of chemotherapy has to be administered. 10. Follow up of patients and acceptance to treatment is low (below 75%) and should be improved. 11. Emphasis on early screening & diagnosis is advised to enhance prognosis and quality of life in these patients. 12. All the treatment modalities for retinoblastoma available in western countries can be made available in tertiary eye care centre in India. Chemotherapy including triple regimen (VEC), Quadruple regimen in resistant tumour, Intrathecal chemotherapy for trilateral retinoblastoma can be safely administered in a tertiary eye care centre & response can be assessed. With the early diagnosis, application of chemo reduction & local therapy the percentage of Enucleation and EBRT, morbidity and mortality of patients can be decreased. 13. There is a necessity to create a national registry to understand the actual impact of retinoblastoma in our country, treatment modalities should be standardized so that every centre can offer best available treatment at lower cost for patient. 14. A multi centered clinical study in India is necessary to evaluate clinical presentation, response of chemoreduction, local therapy in retinoblastoma in India.

Item Type: Thesis (Masters)
Uncontrolled Keywords: Retinoblastoma; demographic features; clinical presentations; treatment modalities; tertiary care centre
Subjects: MEDICAL > Ophthalmology
Depositing User: Devi S
Date Deposited: 15 Feb 2018 07:07
Last Modified: 15 Feb 2018 07:07

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