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

Choi, Sang Yul; Kim, Dong Hwan; Lee, Kang Min; Lee, Hyun Jae; Kim, Mi-Sook; Lee, Tai-Won; Choi, Sang Wook; Kim, Dong Ho; Park, Kyung Duk; Lee, Jun Ah

Original Article

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

최상렬^²^, 김동환^¹^, 이강민^¹^, 이현재^¹^, 김미숙^³^, 이태원^²^, 최상욱^¹^, 김동호^¹^, 박경덕^¹^, 이준아^¹

^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