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Familial retinoblastoma: where and when?
D H Abramson1, M E Mendelsohn, C A Servodidio
1Ophthalmic Oncology Center, New York Hospital-Cornell Medical Center, NY, USA.
Acta Ophthalmologica Scandinavica
|August 1, 1998
Summary
For patients with a family history of retinoblastoma, new tumors typically appear within the first year of life. Tumor location, particularly in the macula, follows a predictable pattern based on age at diagnosis.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Genetics
Background:
- Retinoblastoma is the most common primary intraocular malignancy in children.
- A family history of retinoblastoma increases the risk of developing the disease.
- Early detection and monitoring are crucial for favorable outcomes.
Purpose of the Study:
- To establish the timeline for intraocular disease development in at-risk children.
- To identify the locations where new retinoblastoma tumors typically arise.
- To understand the pattern of tumor occurrence in relation to age and affected eye.
Main Methods:
- Retrospective chart review of retinoblastoma patients.
- Analysis of initial eye examination status and subsequent tumor development.
- Correlation of tumor location with age at diagnosis.
Main Results:
- 62% of first eyes with prior normal exams developed retinoblastoma by 6 months; 90% by 12 months.
- 27% of second eyes were affected by 6 months; 64% by 12 months.
- Younger age at diagnosis correlated with posterior pole tumors; macular tumors appeared early, with no new macular tumors after initial diagnosis.
Conclusions:
- The onset, location, and frequency of new retinoblastoma tumors exhibit a predictable pattern.
- Age at diagnosis is a key factor in determining tumor location.
- Macular tumors present early, and their development appears to cease after initial diagnosis.