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Updated: Aug 22, 2026
Establishment and Propagation of Human Retinoblastoma Tumors in Immune Deficient Mice
Published on: August 4, 2011
Diagnosis and management of retinoblastoma
Carol L Shields1, Jerry A Shields
1Ocular Oncology Service, Wills Eye Hospital, Thomas JeffersonUniversity, Philadelphia, PA 19107, USA. carol.shields@shieldsoncology.com
Insights
Advances in treating retinoblastoma, a childhood eye cancer, have improved survival rates. Newer methods like chemoreduction and focal therapies preserve eyes, reducing the need for enucleation or radiotherapy.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Medical Genetics
Background:
- Retinoblastoma is a malignant eye tumor primarily affecting children under three.
- It poses a significant mortality risk globally, particularly in regions with limited advanced medical care.
Purpose of the Study:
- To review clinical features and diagnostic methods for retinoblastoma.
- To assess current management trends and treatment outcomes.
Main Methods:
- Systematic review of published literature on retinoblastoma.
- Evaluation of clinical data and treatment strategies.
Main Results:
- A global mortality rate of 50% for retinoblastoma contrasts with <5% in developed nations.
- Management trends show a shift from enucleation/radiotherapy to chemoreduction and focal therapies.
- Chemoreduction and focal therapies are highly effective for early-stage retinoblastoma, preserving eyes in over 85% of cases.
Conclusions:
- Significant progress in retinoblastoma diagnosis and management has led to high survival rates.
- Chemoreduction and focal treatments offer eye and visual function preservation, reducing reliance on radiotherapy.
Background:
Retinoblastoma is a highly malignant tumor of the eye that manifests most often in the first 3 years of life.
Methods:
Published articles were reviewed to evaluate the clinical features and current methods of diagnosis and to assess the trends in management.
Results:
This malignancy leads to metastatic disease and death in 50% of children worldwide but in less than 5% of children in the United States and other developed nations with advanced medical care. Over the past decade, there has been a trend away from enucleation and external beam radiotherapy and toward chemoreduction followed by focal therapies. This is largely due to more effective chemotherapeutic regimens, improved focal treatment modalities, and the desire to avoid loss of the globe and/or exposure to radiotherapy. Chemoreduction and focal therapies are most successful for eyes with minimal to moderate retinoblastoma, with enucleation needed in less than 15% of cases. Eyes with very advanced retinoblastoma require enucleation in approximately 50% of cases.
Conclusions:
Progress in the clinical recognition and management of retinoblastoma has led to high survival rates. Improved methods of treatment using chemoreduction and focal treatments without the need for external beam radiotherapy allow preservation of the eye in some cases, often with visual function.
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