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%. 
 Keywords: chemotherapy; outcome; remission; retinoblastoma.

Globe salvaging conservative management of Retinoblastoma

“…3 Developed countries have achieved near zero mortality while developing countries like Nepal still sees high mortality (19%) and morbidity in retinoblastoma patients. 4 In our study male patients were double the number of female patients similar to Congo. 5 Though most studies show male preponderance of disease, studies in Singapore, Iran and New South Wales show female preponderance.…”

Retinoblastoma in Asia

“…Survival rates reported from India were 75.7 to 92% with a mean/median follow-up of 21-44 months [18][19][20][21]. Survival rates reported from less developed countries like Taiwan, Africa, Kenya, and Nepal are 64.41, 57.7 and 26.6%, 23.8%, respectively [11][12][13][14].…”

“…In developed countries, the goal of treatment has shifted from globe salvage to vision preservation [8][9][10]. However, preventing death is a major challenge in under developed nations where most children have advanced disease at presentation [11][12][13][14].…”

Pediatric oncology services in Nepal

“…The cure rate of cancer at KCH is currently around 40%, with 35% abandonment of therapy due to financial burden and 25% mortality (mainly due to infection, drug toxicity, and relapse). [3,4] Cancer programs and support for family Various nongovernmental organizations (NGOs) have been established to improve cancer awareness and prevention in Nepal, including the Nepal Cancer Relief Society, the Cancer Society Nepal, and Cancer Care Nepal. [5] There are a few NGOs helping children with cancer in Nepal, but the focus is on treating adults with cancer.…”