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Second primary osteosarcomas in patients with retinoblastoma
Tomohiro Fujiwara1, Miyuki Fujiwara2, Kunihiko Numoto1
1Division of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo.
Japanese Journal of Clinical Oncology
|October 7, 2015
Summary
Second primary osteosarcomas are a concern for retinoblastoma survivors. Limb osteosarcomas in survivors show promising outcomes with modern chemotherapy and surgery.
Area of Science:
- Oncology
- Ophthalmology
- Pediatric Oncology
Background:
- Second primary malignancies are the leading cause of death in retinoblastoma survivors.
- Osteosarcoma is the most common second malignancy, but its characteristics are poorly understood.
Purpose of the Study:
- To investigate the clinical and therapeutic features of osteosarcoma as a second malignancy in retinoblastoma survivors.
- To evaluate treatment outcomes for these patients.
Main Methods:
- Retrospective review of a database of retinoblastoma patients who developed osteosarcoma between 1964 and 2010.
- Analysis of patient demographics, treatment modalities, and outcomes.
Main Results:
- 1.1% (10/857) of retinoblastoma patients developed osteosarcoma.
- Median age at osteosarcoma onset was 11.2 years; 4 were radiation-related, 6 were extremity-based.
- Four extremity osteosarcomas treated with surgery and chemotherapy showed a 75% survival rate with no evidence of disease.
Conclusions:
- Osteosarcoma in the extremity of retinoblastoma survivors may have a better prognosis than conventional osteosarcoma.
- Early detection of radiation-related craniofacial osteosarcoma is crucial for successful resection.
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