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Retinoblastoma: ten years experience at Kanti Children's Hospital
Kailash Prasad Sah1, Rohit Saiju2, Pankaj Roy1
1Kanti Children's Hospital, Kathmandu, Nepal.
Insights
This study analyzed retinoblastoma cases over ten years, revealing a 23.8% survival rate despite resource limitations. Key findings include common presenting signs and tumor differentiation in pediatric patients with this eye cancer.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Cancer Research
Background:
- Retinoblastoma is the most common childhood intraocular malignancy, primarily affecting children under 4 years old.
- It is a disease of early childhood with a median diagnosis age of 2 years, and incidence rates around 1 in 15,000-18,000 live births.
Purpose of the Study:
- To investigate the clinical pathological features of retinoblastoma.
- To analyze treatment protocols and survival outcomes in children diagnosed with retinoblastoma over a decade.
Main Methods:
- A retrospective hospital-based study was conducted from March 1998 to February 2008.
- Data collected included patient demographics, clinical presentation, pathology reports, treatment strategies, and follow-up outcomes.
Main Results:
- 42 children with retinoblastoma were studied; 85.7% had unilateral disease, and 50% showed optic nerve involvement.
- Common presentations included extraocular signs (28.6%), proptosis (23.8%), and leukoria (23.8%). Majority had poorly differentiated tumors (62%).
- The overall 10-year survival rate was 23.8%, with 19% mortality and 57.2% lost to follow-up or left against medical advice.
Conclusions:
- Despite significant resource limitations, the pediatric oncology unit achieved a 23.8% success rate in treating retinoblastoma.
- The study highlights challenges in managing retinoblastoma, particularly regarding patient follow-up and treatment adherence in resource-limited settings.
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%.