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http://dx.doi.org/10.3345/kjp.2009.52.6.674

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

Choi, Sang Yul (Department of Ophthalmology, Korea Cancer Center Hospital)
Kim, Dong Hwan (Department of Pediatrics, Korea Cancer Center Hospital)
Lee, Kang Min (Department of Pediatrics, Korea Cancer Center Hospital)
Lee, Hyun Jae (Department of Pediatrics, Korea Cancer Center Hospital)
Kim, Mi-Sook (Department of Radiation Oncology, Korea Cancer Center Hospital)
Lee, Tai-Won (Department of Ophthalmology, Korea Cancer Center Hospital)
Choi, Sang Wook (Department of Pediatrics, Korea Cancer Center Hospital)
Kim, Dong Ho (Department of Pediatrics, Korea Cancer Center Hospital)
Park, Kyung Duk (Department of Pediatrics, Korea Cancer Center Hospital)
Lee, Jun Ah (Department of Pediatrics, Korea Cancer Center Hospital)
Publication Information
Clinical and Experimental Pediatrics / v.52, no.6, 2009 , pp. 674-679 More about this Journal
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 $56.8{\pm}7.5%$. 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;
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