Bilateral retinoblastoma: Long-term follow-up results from a single institution.
10.3345/kjp.2009.52.6.674
- Author:
Sang Yul CHOI
1
;
Dong Hwan KIM
;
Kang Min LEE
;
Hyun Jae LEE
;
Mi Sook KIM
;
Tai Won LEE
;
Sang Wook CHOI
;
Dong Ho KIM
;
Kyung Duk PARK
;
Jun Ah LEE
Author Information
1. Department of Ophthalmology, Korea Cancer Center Hospital, Korea.
- Publication Type:Original Article
- Keywords:
Bilateral retinoblastoma;
Long-term effects;
Treatment
- MeSH:
Cataract;
Eye;
Facial Asymmetry;
Follow-Up Studies;
Humans;
Medical Records;
Neoplasms, Second Primary;
Recurrence;
Retinal Detachment;
Retinoblastoma;
Retrospective Studies;
Survival Rate
- From:Korean Journal of Pediatrics
2009;52(6):674-679
- CountryRepublic of Korea
- Language:English
-
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.
