Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Effects of enucleation and chemotherapy in advanced intraocular and intraorbital retinoblastoma with or without radiotherapy

진행된 안구내 및 안와내 망막모세포종에서 안구적출술과 항암화학치료 및 방사선조사 유무에 따른 효과

  • Lee, Jae Min (Department of Pediatrics, College of Medicine, Yeungnam University) ;
  • Lee, Hyun Dong (Department of Pediatrics, College of Medicine, Yeungnam University) ;
  • Hah, Jeong Ok (Department of Pediatrics, College of Medicine, Yeungnam University)
  • 이재민 (영남대학교 의과대학 소아과학교실) ;
  • 이현동 (영남대학교 의과대학 소아과학교실) ;
  • 하정옥 (영남대학교 의과대학 소아과학교실)
  • Received : 2007.10.11
  • Accepted : 2007.11.25
  • Published : 2008.01.15
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Abstract

Purpose : Radiotherapy is effective in local treatment for retinoblastoma. However, asymmetric facial hypoplasia after radiation is a serious late effect. This study was performed to investigate the effects of enucleation and chemotherapy with or without radiotherapy in advanced intraocular and intraorbital retinoblastoma. Methods : Between 1985 October and 2006 December, the records of thirty five patients who were diagnosed as retinoblastoma at Yeungnam University Hospital were reviewed. Advanced intraocular and intraorbital retinoblastoma patients classified as Reese-Ellsworth group III, IV, and V and Grabowski- Abramson class II were selected for the study. Results : Eighteen patients were enrolled in this study. All patients were enucleated and had received chemotherapy. Nine patients received radiotherapy and nine patients didn't receive radiotherapy. Tumor cells were found on resection margin of optic nerve in five of nine patients who received radiotherapy, but none of nine who didn't receive radiotherapy. Chemotherapy included vincristine, adriamycin, cyclophosphamide, VM-26, cisplatin before 2001, and vincristine, etoposide, and carboplatin after 2001. There were no recurrences or metastases in nine patients who didn't receive radiotherapy. But two of nine patients who received radiotherapy had metastases to brain. However, all survivors who received radiotherapy had significant facial asymmetry. Conclusion : In advanced intraocular and intraorbital retinoblastoma without tumor cell on resection margin of optic nerve, enucleation and chemotherapy without local radiotherapy appears to be safe for long-term survival. However, in those with tumor cells on resection margin of optic nerve, enucleation and chemotherapy with local radiotherapy seems to be necessary to improve survival.

목 적 : 방사선조사는 망막모세포종의 국소적 치료에 효과적인 방법이나 장기 생존자에서 안면발육장애, 각막과 시신경 손상, 이차적인 암의 발생 등의 합병증을 유발하며 방사선조사로 인한 안면발육부전은 나이가 들수록 더욱 심각한 미용적 장애를 초래하고 환자의 삶의 질을 저하시킨다. 진행된 안구 내 망막모세포종과 안와 내 망막모세포종 환자에서 안구적출술과 항암화학치료 및 안와방사선조사를 받은 경우와 받지 않은 경우의 치료성적과 장기예후를 분석하고자 본 연구를 시도하였다. 방 법 : 1985년 10월부터 2006년 12월까지 영남대학교병원에서 망막모세포종으로 진단받은 35명의 환자를 대상으로 병록지를 후향적으로 분석하였다. 안구 내 병변은 Reese-Ellsworth(RE)의 분류에 따라, 안구 외로 진행된 경우는 Grabowski-Abramson (GA)의 분류에 따라 분류하여 RE group III, IV, V인 진행된 안구 내 종양과 GA stage II인 안와 내 종양 환자를 연구 대상으로 하였다. 결 과 : 연구에 포함된 환자는 18명이었고, 진단당시 나이의 중앙값은 27개월(범위 2-69개월), 추적기간의 중앙값은 73.5개월(범위 6-219개월)이었다. 안구 내에 국한된 경우는 6명이었고(RE group III; 2명, IV; 1명, V; 3명) 안와 내로 진행된 경우는 12명이었다(GA stage II). 대상 환아 모두에서 안구적출술을 시행한 후 항암화학치료를 시행하였는데 2001년 이전에는 vincristine, adriamycin, cyclophosphamide, cisplatin, VM-26을, 2001년 이후로는 vincristine, etoposide, carboplatin을 사용하였다. 9명의 환자에서는 국소적으로 방사선조사를 병행하였고, 총 방사선 조사량의 중앙값은 4,500 cGy(범위 3,500-5,000 cGy)이었다. 방사선조사를 병행하여 치료 받은 환아 9명 중 7명은 재발이나 전이 없이 장기생존하였으며, 장기 생존자 모두에서 심한 안면 골격의 비대칭적 발육부전이 나타났다. 방사선조사 없이 치료받은 9명 중에서는 재발이나 전이가 한명도 없이 모두 장기생존하였으며, 안구적출술 이후 인공안와삽입물을 삽입하였으며 안면 골격 발달은 모두 대칭적으로 정상 발육을 하였다. 결 론 : 시신경의 절제면을 침범하지 않은 안와내에 국한된 망막모세포종의 치료에 있어 방사선조사를 제외하고 안구적출술, 항암화학치료, 국소적 치료를 병행함으로써 높은 생존율을 유지하면서 안면발육부전과 같은 심각한 부작용을 줄여 삶의 질을 향상시킬 수 있을 것으로 생각된다. 그러나 시신경의 절제면을 침범한 경우에는 재발의 가능성이 있으므로 후기 후유증의 위험이 있더라도 안구적출술 후 방사선조사와 항암화학치료를 병행한 치료를 시행함으로써 생존률을 높일 수 있을 것으로 생각된다.

Keywords

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