Incidence, ocular manifestations, and survival in children with neuroblastoma: a population-based study.
OBJECTIVE
To determine the incidence, ophthalmic manifestations, and survival among children with neuroblastoma in a defined population.


METHODS
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.
Smith
Stephen J
SJ
Mayo Clinic College of Medicine, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.
Diehl
Nancy N
NN
Smith
Brian D
BD
Mohney
Brian G
BG
D000008:Abdominal Neoplasms / Q000453:epidemiology* / Q000473:pathology; D000310:Adrenal Gland Neoplasms / Q000453:epidemiology* / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008479:Mediastinal Neoplasms / Q000453:epidemiology* / Q000473:pathology; D008910:Minnesota / Q000453:epidemiology; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000453:epidemiology* / Q000556:secondary; D009918:Orbital Neoplasms / Q000453:epidemiology* / Q000556:secondary; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate