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Outcome after Treatment of Retinoblastoma

Journal of the Korean Pediatric Society 1997;40(4):519-530.
Published online April 15, 1997.
Outcome after Treatment of Retinoblastoma
Hyoung Soo Choi1, Ki Woong Seong1, Jae Kyung Lee1, Kyung Ha Yoo1, Hee Young Shin1, Young Suk Yu2, Hyo Seop Ahn1
1Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
2Department of Ophthalmology, Seoul National University, College of Medicine, Seoul, Korea
망막모세포종의 항암치료성적
최형수1, 성기웅1, 이재경1, 유경하1, 신희영1, 유영석2, 안효섭1
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 안과학교실
Abstract
Purpose
: Current treatment in retinoblastoma aims to save life and preserve useful vision by using combined modality therapy. This study was performed to determine the outcome after treatment of retinoblastoma.
Methods
: Medical records of all cases of retinoblastoma seen at Seoul National University Children's Hospital from September 1986 to April 1995 were reviewed. During this period, the treatment method was consistent for the patients with retinoblastoma as follows; for intraocular disease with visual potential, nonsurgical treatment consisted of radiation therapy and/or light coagulation, cryotherapy was considered, followed by enucleation if there was no response, and for intraocular disease without visual potential and extraocular disease, enucleation of those eyes that had severe involvement followed by radiation therapy and/or chemotherapy(systemic or intrathecal) was done according to the clinical pathologic staging.
Results
: In 58 patients, 6 patients died and the 3-year survival rate was 83%. There was no difference in survival rate between 47 unilateral and 11 bilateral cases, which were 80% and 91%, respectively(P=0.790). The survival rate of 74% for 16 extraocular disease was lower than that of 87% for 42 intraocular disease and the difference of the survival rate between these two groups has shown borderline statistical significance (P=0.056). The 3-year event free survival rate was 69% in overall patients. Complications following therapy included infection due to bone marrow suppression, cardiomyopathy, and orbital contracture. One case of osteosarcoma has developed as a second malignant neoplasm.
Conclusion
: To improve the survival of retinoblastoma, effective therapy for extraocular disease and prevention of extraocular extension are important. Supportive care to minimize the complications of therapy and long-term follow-up for second malignant neoplasm are also needed.
Key Words: Retinoblastoma, Combined modality therapy, Survival rate


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