Related Experiment Videos
Trilateral retinoblastoma--incidence and outcome: a decade of experience
L E Blach1, B McCormick, D H Abramson
1Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
Insights
Trilateral retinoblastoma, a rare cancer in children with bilateral retinoblastoma, is a significant cause of death. Early screening of high-risk survivors is crucial for improved outcomes.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Radiation Oncology
Background:
- Retinoblastoma is a common childhood eye cancer.
- Trilateral retinoblastoma involves the eye and the brain's pineal gland.
- Bilateral retinoblastoma and family history increase risk.
Purpose of the Study:
- To determine the incidence and outcomes of trilateral retinoblastoma.
- To identify patient groups at high risk for trilateral retinoblastoma.
- To evaluate mortality associated with trilateral retinoblastoma.
Main Methods:
- Retrospective review of 117 children treated for retinoblastoma (1979-1990).
- Median follow-up of 68 months.
- Analysis of trilateral retinoblastoma incidence and treatment outcomes.
Main Results:
- Six cases of trilateral retinoblastoma were identified (6% incidence in bilateral cases).
- Trilateral retinoblastoma occurred at a younger median age (3 months) than retinoblastoma.
- All patients with trilateral retinoblastoma died, accounting for 50% of deaths in the cohort.
Conclusions:
- Trilateral retinoblastoma is an under-recognized cause of mortality in retinoblastoma survivors.
- Aggressive screening of high-risk retinoblastoma patients is warranted.
- Early detection and intervention strategies are needed.
Purpose:
This report examines the incidence and outcome of trilateral retinoblastoma in children treated for retinoblastoma. A group of patients who are at highest risk for the development of trilateral retinoblastoma is defined.
Methods And Materials:
Between 1979 and 1990, 117 children were treated with external beam radiation therapy for retinoblastoma, (97/117, bilateral). Median follow-up time was 68 months. The median age at diagnosis was 7 months.
Results:
Six cases of trilateral retinoblastoma were identified. The incidence of trilateral retinoblastoma in children with bilateral retinoblastoma was 6% (6/97) and 10% in those with a family history of retinoblastoma. The median age at diagnosis of RB in the children with trilateral retinoblastoma, was 3 months, younger than the median age of the entire retinoblastoma group. In all cases, the pineal region was excluded from the radiotherapy fields. Treatment for the trilateral retinoblastoma consisted of craniospinal axis radiation therapy and chemotherapy in three patients, chemotherapy alone in two, and no treatment in one. All patients died from this disease. Overall, of the 117 children treated at our institution for retinoblastoma with a median follow-up of 68 months, 12 have died. Trilateral retinoblastoma was the major cause of death, accounting for 50% (6/12) of deaths.
Conclusion:
Trilateral retinoblastoma is a major and under-appreciated cause of mortality in the first 5 years after the diagnosis of bilateral retinoblastoma. A more aggressive approach toward screening a defined population of childhood retinoblastoma survivors may be warranted.