목적 : 양안 망막모세포종 환아 중 양안 안구적출을 받은 환아의 임상경과를 알아보고자 하였다. 대상과 방법 : 1996년부터 2005년까지 양안 망막모세포종으로 진단하여 치료를 시행한 48명의 환자 중에서 양안 안구적출을 받았던 환아 5명의 진단 시 종양 상태, 치료 경과, 양안 안구적출술의 이유를 후향적으로 분석하였다. 결과 : 양안 망막모세포종 환아 48명 중 양안을 모두 보존한 경우가 6명, 1안을 보존한 경우가 37명, 양안을 모두 적출한 경우가 5명이었다. 양안을 적출한 환아 5명 모두 여자였고, 가족력은 없었다. 진단시 나이는 3명은 4개월, 2명은 각각 1세, 1.5세였다. 종양 상태는 3명은 양안 Reese-Ellsworth (R-E) 분류 V였고, 2명은 한눈이 Ⅲ, 반대편 눈이 V이었다. 첫 치료방법으로 단안 안구적출술 후 항암치료를 시행한 경우가 3명, 일차적 항암치료를 시행한 경우가 2명이었다. 추가치료로 레이저 광응고술, 냉동치료, 외부방사선조사 또는 양전자치료를 병용하였다. 이러한 치료에도 불구하고 새로운 종양이 다발성으로 발생하거나, 기존 종양이 다시 자라거나, 유리체 퍼짐이 심하게 진행하여 안구적출술을 추가로 시행하였다. 결론 : 양안 안구적출술을 시행받은 환아들은 대부분 1세 이전의 어린 연령에서 진단을 받았고, 진단시부터 높은 R-E 병기를 보였으며, 집중적인 치료에도 반응이 불량한 경우가 많았다.

Purpose: To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients. Methods: Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation. Results: Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation. Conclusion: Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.

Purpose: To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients. Methods: Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation. Results: Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation. Conclusion: Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.

Retinoblastoma, Enucleation, Bilateral retinoblastoma, Bilateral enucleation