Incidence, Ocular Manifestations, and Survival in Children with Neuroblastoma: A Population-Based Study

Purpose: To determine the incidence, ophthalmic manifestations, and survival among children with neuroblastoma in a defined population. Design: Population-based retrospective cohort. Methods: The medical records of all pediatric (<19 years) residents of Olmsted County, Minnesota, diagnosed with neuroblastoma from January 1, 1969, through December 31, 2008, were retrospectively reviewed. Results: Fourteen children were diagnosed with neuroblastoma as residents of Olmstead County, Minnesota, during the 40-year period, yielding an age- and gender-adjusted incidence of 11.8 (95% confidence interval [CI]: 5.6-18.0) per million patients <15 years of age. The calculated incidence for patients presenting before the age of 5 in this cohort was 1 in 5970 children (95% CI: 3920-12 580 children). The mean age at diagnosis for the 14 study patients was 22.5 months (range, 10.4-42.6 months). Six of the 14 (43%; 95% CI: 18%-71%) had ocular manifestations, including orbital metastasis in 6 (100%), proptosis and ecchymosis in 4 (67%), ptosis in 2 (33%), and strabismus in 1 (17%). The Kaplan-Meier rate of survival for all 14 children was 57% at 1 year (95% CI: 36%-90%) and 50% at 5 years (95% CI: 30%-84%), while the 6 with eye findings had a survival rate of 17% at 9 months (95% CI: 3%-100%). Conclusions: The incidence of neuroblastoma in this population was 11.8 per million patients <15 years, with ophthalmic involvement observed in 6 of the 14 study patients (43%). Orbital metastasis in the 6 children in this cohort was associated with poor prognosis.