Retinoblastoma: Ten years experience at Kanti children's hospital

Abstract

Introduction: Retinoblastoma is a malignant tumor of the embryonic neural retina. It is the most common intraocular tumor of childhood. It is a disease of early childhood with 80% cases diagnosed before the age of 4 years. The median age at diagnosis is 2 years. Bilateral cases are diagnosed earlier (median at 12 months and unilateral median age at 24 months). The incidence of retinoblastoma is approximately 1 in 15000-18000 live births in developed countries. The aim of the research is to study the clinical pathological features, treatment and survival of children with retinoblastoma over ten years. Methods: A retrospective hospital based study was conducted from March 1998 to February 2008. Gender, age, clinical presentation, pathology reports, treatment, follow -up and outcome were recorded at time of diagnosis. Results: A total of 42 children with retinoblastoma (85.7 % unilateral, 14.3% bilateral), with 50% having optic nerve involvement were studied. The most common presenting signs were extraoccular (28.6%), proptosis (23.8%), leukoria (23.8%), phthisis bulbi (16.7%), and strabismus (7.1%). The age at presentation ranged from 6-120 months with mean age of 46.6 months. The male to female ratio was 1.1:1.Two-thirds presented between 2-5 yrs, followed by 12-24 months (23.8%). Majority of children had poorly differentiated retinoblastoma (62 %), followed by well differentiated (28.6%), and moderately differentiated (9.5%). CEV based protocol used to treat, and over-all 10 yrs survival was 23.8 %, death 19 %, and lost to follow-up or left against medical advice 57.2%. Conclusions: Despite severe resource limitation, pediatric oncology unit has been successfully treating retinoblastoma with the success rate of 23.8%.