Korean J Pediatr.  2009 Jun;52(6):674-679. 10.3345/kjp.2009.52.6.674.

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

• Choi SY
• Kim DH
• Lee KM
• Lee HJ
• Kim MS
• Lee TW
• Choi SW
• Kim DH
• Park KD
• Lee JA

• KMID: 2281072
• DOI: http://doi.org/10.3345/kjp.2009.52.6.674

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.

Keyword

Bilateral retinoblastoma; Long-term effects; Treatment

MeSH Terms

Cataract
Eye
Facial Asymmetry
Follow-Up Studies
Humans
Medical Records
Neoplasms, Second Primary
Recurrence
Retinal Detachment
Retinoblastoma
Retrospective Studies
Survival Rate