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Bilateral retinoblastoma: Long-term follow-up results from a single institution

Korean Journal of Pediatrics 2009;52(6):674-679.
Published online June 15, 2009.
Bilateral retinoblastoma: Long-term follow-up results from a single institution
Sang Yul Choi2, Dong Hwan Kim1, Kang Min Lee1, Hyun Jae Lee1, Mi-Sook Kim3, Tai-Won Lee2, Sang Wook Choi1, Dong Ho Kim1, Kyung Duk Park1, Jun Ah Lee1
1Department of Pediatrics, Korea Cancer Center Hospital, Korea
2Department of Ophthalmology, Korea Cancer Center Hospital, Korea
3Department of Radiation Oncology, Korea Cancer Center Hospital, Korea
단일기관의 장기추적 결과
최상렬2, 김동환1, 이강민1, 이현재1, 김미숙3, 이태원2, 최상욱1, 김동호1, 박경덕1, 이준아1
1원자력병원 소아과청소년과
2원자력병원 안과
3원자력병원 방사선종양학과
Correspondence: 
Jun Ah Lee, Email: junahlee@kcch.re.kr
Abstract
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.
Key Words: Bilateral retinoblastoma, Long-term effects, Treatment


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