Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Bilateral retinoblastoma: Long-term follow-up results from a single institution

단일기관의 장기추적 결과

  • 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)
  • 최상열 (원자력병원 안과) ;
  • 김동환 (원자력병원 소아과청소년과) ;
  • 이강민 (원자력병원 소아과청소년과) ;
  • 이현재 (원자력병원 소아과청소년과) ;
  • 김미숙 (원자력병원 방사선종양학과) ;
  • 이태원 (원자력병원 안과) ;
  • 최상욱 (원자력병원 소아과청소년과) ;
  • 김동호 (원자력병원 소아과청소년과) ;
  • 박경덕 (원자력병원 소아과청소년과) ;
  • 이준아 (원자력병원 소아과청소년과)
  • Received : 2009.01.13
  • Accepted : 2009.05.14
  • Published : 2009.06.15
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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.

목 적 : 양측성 망막모세포종 환자들의 치료 후 장기후유증을 분석하고자 하였다. 방 법 : 1987년 10월부터 1998년 10월까지 원자력병원에서 치료를 받고 10년 이상 장기추적관찰 중인 양측성 망막모세포종 환자 22명의 의무기록을 분석하였다. 진단시 연령, 치료지연, Reese-Ellsworth (RE) 분류, 국소치료방법 등의 임상 변수와 생존율, 안구합병증을 포함한 장기후유증의 발생과의 관계를 알아보았다. 결 과 : 진단 시 정중 연령은 7.0 개월로(범위, 1.7-31.6개월), 백색동공으로 내원한 경우가 가장 많았다. 44개 안구의 RE 분류는 각각 group II (n=4), III (n=14), IV (n=4), V (n=22)이었다. 추적관찰 141개월째인 현재 20명이 생존하여 있으며, 10년 안구생존율은 $56.8{\pm}7.5%$이었다. 임상변수 중, 3개월 이상의 치료지연이 재발과 관련이 있었다. 방사선 치료 후 백내장, 망막박리, 안구로, 안면비대칭 등의 합병증이 관찰되었으며 현재까지 이차성 종양의 발생은 없었다. 결 론 : 안구생존율을 향상시키기 위해서는 종양을 조기에 발견하도록 노력하고 진단즉시 치료를 시작해야 할 것이다. 이와 더불어 장기적인 후유증의 발생여부에 대해서도 세심한 추적관찰이 필요하다.

Keywords

Acknowledgement

Supported by : Korea Institute of Radiological and Medical Sciences

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