Outcome after Treatment of Retinoblastoma

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. CONCLUSIONS: 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.