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World Caner Day 2014 Understanding Retinoblastoma
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World Caner Day 2014 Understanding Retinoblastoma


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Retinoblastoma is the most common cause of childhood ocular cancer across the world.

Retinoblastoma is the most common cause of childhood ocular cancer across the world.

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  • 1. Retinoblastoma. factors affecting survival &outcome the “Sankara” Experience Dr Kaushik Murali 1 Dr Vidya C 1 Dr Rajesh R 1 Dr Mahesh P Shanmugam1 Achyut Patil 2 1 Sankara Eye Hospital, Bangalore 2 Yale University, New Haven, USA Retinoblastoma Survival
  • 2. Introduction Retinoblastoma most common intraocular malignancy in children, & reported to affect 1 in 15,000 to 1 in 18,000 live births. Studies from India show a 2-3 fold higher incidence of tumors of the eye. Survival rates for retinoblastoma patients have increased dramatically over the last century with prompt medical care, with documented 5-year survival figures reaching 87-99% in developed countries “Krishnarpanam” – Naraindas Bhudrani - Sankara Ocular Oncology Department establsihed in 2008 to provide comprehensive treatment for Ocular Cancers – Chemotherpoay, Ru Brachytherapy, Surgical Care, LASERs and Ocularistry. Abramson DH. Retinoblastoma incidence in the United States. Arch Ophthalmol. 1990;108:1514 Arora RS, Eden T, Kapoor G. Epidemiology of childhood cancer in India. Indian J Cancer.2009;46:264–73 Kim JW, Current management strategies for intraocular retinoblastoma. Drugs. 2007;67(15):2173-2185 Retinoblastoma Survival
  • 3. Aim of the Study To analyze factors influencing morbidity and mortality in children presenting retinoblastoma at a tertiary referral eye hospital in India. Retinoblastoma Survival
  • 4. Materials & Methods Retrospective review 118 Children treated for retinoblastoma at Sankara Eye Hospital Bangalore, India. Between June 2008 – July 2013. Age at presentation, stage of disease (ABC classification) treatment given studied with outcomes Statistical analysis was done using Microsoft Excel and SPSS software Retinoblastoma Survival
  • 5. Results GENDER NUMBER OF PATIENTS 40% 60% Male 80 60 40 20 0 <5 76 Series1 NUMBER OF PATIENTS Average age of presentation 5.14 years (1 -18 yrs). Bilateral Disease in 58 (49%). 17 (14.4%) mortality was noted at last followup. 9(7.6%) children were lost follow-up. Late recurrence -17 (14.4%) among which 82% expired. 4 developed secondary radiation cataract. Age of Presentation `6-10 32 >10 7 TREATMENT MODALITIES 80 60 40 20 0 Multimodal Enucleation 59 46 Series1 Chemothera py 6 Self regressed 1 Retinoblastoma Survival
  • 6. Factor Significance on Survival Inference Age Sex p=0.67 p=0.55 Not Significant Not Significant Presenting Eye (right/left) Stage of disease p=0.67 Not Significant Group D p=0.053 Group E p=0.004 p=0.036 P=0.00026 Significant latency > 6 months Recurrence Significant Significant Retinoblastoma Survival
  • 7. Discussion Retinoblastoma represents almost 4% of all paediatric malignancies. 95% children with retinoblastoma survive in developed world, 50% survive worldwide. Combination of poverty, illiteracy, alternative systems of medicine and lack of access to healthcare resources account for this high rate of advanced disease in developing countries. Abramson DH. Retinoblastoma incidence in the United States. Arch Ophthalmol. 1990;108:1514 Leal-Leal C. A multicentre report from the Mexican Retinoblastoma Group. Br J Ophthalmol.2004;88:1074–7 Rachna Meel. Current therapy and recent advances in the management of retinoblastoma. Indian J Med Paediatr Oncol. 2012 Apr-Jun; 33(2): 80–88. Retinoblastoma Survival
  • 8. Current Study India Study North America & Europe Unilateral Retinoblastoma 38 months 30 months 24 months Bilateral Retinoblastoma 19 months 24 months Male:Female 1.4:1 1.6:1 1:1 Laterality % 51% unilateral 70 % unilateral 70% unilateral Leucocoria Leucocoria Leucocoria 73:27 60:35 90:10 Median Age of Presentation Most common presentation Intraocular: advanced/metastatic disease Bakhshi S, Gupta S, Gogia V, Ravindranath Y. Compliance in retinoblastoma. Indian J Pediatr.2010;77:535–40 Aerts I. Retinoblastoma.Orphanet J Rare Dis. 2006;25:1–31. Gombos DS. Estimating the incidence of retinoblastoma in Texas. Tex Med. 2005;101:70–2. Abramson DH.Presenting signs of retinoblastoma. J Pediatr. 1998;132:505–8 Retinoblastoma Survival
  • 9. • Provision of comprehensive care including all modalities and free care enhanced compliance. • With multimodal treatment anatomic integrity could be preserved in atleast one eye in 98.3% • Advanced Disease (Group D&E) and increased latency > 6 months proved detrimental. • Recurrence was seen 14 months ( 8-26months). 82% children with recurrent lost their life. • Adjuvant chemotherapy in children with massive choroidal invasion and cut end involvement (Pathological staging of >pT3c) at presentation following enucleation did not have beneficial role in preventing mortality.This coincided more with TNM pathological staging rather than international ABC staging. Though sample is small for statistical significance. • Retrospective review limits data correlation & short follow up may have impact on survival. An analysis of causes of recurrence, matastasis and death of retinoblastoma].Yan Ke Xue Bao. 1995 Dec ;11(4):221-3 Survival in extra-orbital metastatic retinoblastoma:treatment results.Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J.Clin Transl Oncol. 2006 Jan; 8(1):39-44 Retinoblastoma Survival
  • 10. Conclusion Outcomes of retinoblastoma can be improved with early recognition and intervention. Late recurrence is a possibility in Indian children and these children need long term follow up. Retinoblastoma Survival
  • 11. Thank You For further information, please write to: Retinoblastoma Survival