Retinoblastoma treated in infants in the first six months of life.

Overview

One hundred fifty-eight children with retinoblastoma received diagnoses and were treated during the first six months of life and then were observed for a mean of 7.4 years. The mean age at diagnosis was 3.6 months. Four patients received diagnoses at birth, one of metastatic disease. Ninety-four patients (60%) had leukokoria, reflecting the fact that 108 (68%) were initially seen with group V tumors (Reese-Ellsworth staging) despite their young age. One hundred seven (68%) were initially seen with bilateral retinoblastoma and 51 (32%) were seen with unilateral disease; in ten of the latter group, tumors later developed in the fellow eye. The eyes of 90 patients with bilateral involvement were asymmetric at first visit (that is, their eyes were involved at different stages of disease). Of 52 eyes receiving bilateral irradiation, 26 (50%) required further treatment, 23 (70%) of these for a resistant tumor and ten (30%) for new tumors. Despite the early age at diagnosis and treatment, 13 (25%) of treated eyes ultimately required enucleation. Metastatic retinoblastoma developed in ten patients and they died. Second, nonocular tumors developed in 13 patients. Early age at diagnosis does not ensure that the disease is caught at an early stage.