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KMID : 1130320090520060674
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Korean Journal of Pediatrics
2009 Volume.52 No. 6 p.674 ~ p.679
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Bilateral retinoblastoma: Long-term follow-up results from a single institution
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Choi Sang-Yul
Kim Dong-Hwan
Lee Kang-Min
Lee Hyun-Jae
Kim Mi-Sook
Lee Tai-Won
Kim Dong-Ho
Park Kyung-Duk
Lee Jun-Ah
Choi Sang-Wook
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Abstract
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Purpose : The authors aimed to analyze the long-term effects of treatments, especially external beam radiotherapy (EBRT), in bilateral retinoblastoma patients.
Methods : This retrospective study analyzed the medical records of 22 bilateral retinoblastoma patients who were registered between October, 1987 and October, 1998 and followed-up for more than 10 years. They were treated by enucleation, EBRT, and systemic chemotherapy. Age at diagnosis, sex, delay prior to treatment, Reese-Ellsworth (RE) classification, and the local treatment modalities were analyzed in relation to recurrence-free survival (RFS) and complications.
Results : Median age at diagnosis was 7.0 months (range 1.7-31.6 months). Leukocoria was the most common presenting feature. Two patients had a familial history. The RE classifications of the 44 eyes were group II in 4, III in 14, IV in 4, and V in 22. At the end of a median follow-up period of 141 months (range 55-218 months), 20 patients were alive. The 10-year ocular survival rate of the 44 eyes was 56.8±7.5%. The 10-year RFS and ocular survival rate of the 29 eyes treated by combined EBRT and chemotherapy were 75.9% and 86.2%, respectively. Treatment delay (＞3 months) was found to be related to higher risk of recurrence. Complications after EBRT were cataract, retinal detachment, phthisis bulbi, and facial asymmetry. No patient developed a second malignancy during the follow-up period.
Conclusion : Early detection and prompt treatment can increase ocular survival rates. In addition, careful attention should be paid to possible long-term sequelae in these patients.
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KEYWORD
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Bilateral retinoblastoma, Long-term effects, Treatment
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