A Clinical Study on Retinoblastoma

Choi, Sang Wook

Abstract

PURPOSE: Retinoblastoma is the most common primary intra-ocular tumor in children. This study was done to evaluate the clinical characteristics and treatment outcome of retinoblastoma in Korea Cancer Center Hospital. METHPDS: Forty five retinoblastoma patients admitted in Korea Cancer Center Hospital from November 1987 to April 1999 were studied.
RESULTS
Age at diagnosis was one month to 84 months after birth, and the median age was 19 months for unilateral disease and 12 months for bilateral disease. Male to female ratio was 2.2:1. Unilateral retinoblastoma was 42.2%, and bilateral retinoblastoma was 57.8%. Two patients with bilateral disease had family history of retinoblastoma inherited by father. Chief complaints were leukokoria 53.3%, strabismus 22.2%, orbit swelling 6.7%, orbit mass 6.7%, ocular protrusion, headache, white opacity, red eye, eye discharge, 2% each. Metastasis at diagnosis to brain 6, orbit and bone 5, optic nerve 3, bone marrow 2, vitreous body 1 was found. Five cases of relapse during treatment were found, and three of them were dead. Seven of 45 patients were dead. Five year overall survival rate was 81.4% (stage I 96.7%, stage II 66.7%, stage III 50%, stage IV 50%). Fever and neutropenia due to myelosuppression was the most common complication of the treatment. Radiation induced complication such as cataract, facial bone asymmetry needed further supportive management. No secondary neoplasm has been found so far.
CONCLUSION
Retinoblastoma is a curable disease of overall survival rate of over 90% with early diagnosis and accurate treatment. But the prognosis is dismal if CNS is involved. Combination of new chemotherapeutic agents or high dose chemotherapy with stem cell rescue could improve the overall survival. Further trials to prevent enucleation are highly recommended.