All issues > Volume 52(6); 2009

Original Article

Korean J Pediatr. 2009;52(6):674-679. Published online June 15, 2009.

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

Sang Yul SY Choi², Dong Hwan DH Kim¹, Kang Min KM Lee¹, Hyun Jae HJ Lee¹, Mi-Sook MS Kim³, Tai-Won TW Lee², Sang Wook SW Choi¹, Dong Ho DH Kim¹, Kyung Duk KD Park¹, Jun Ah JA 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

Correspondence Jun Ah JA 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.

Keywords :Bilateral retinoblastoma, Long-term effects, Treatment