Factors Associated With the Improved Survival of Head and Neck Neuroblastomas Compared to Other Body Sites

To examine pediatric neuroblastoma survival and management in the head and neck compared to other body sites. Retrospective analysis of a large population database. Patients in the Surveillance, Epidemiology, and End Results (SEER) database with neuroblastoma, NOS; ganglioneuroblastoma; or olfactory neuroblastoma diagnosed from birth to 18 years between 1973 and 2014 were included. These patients were classified into 1 of 3 categories based on primary tumor site: head and neck, adrenal, and “other.” Four thousand five hundred neuroblastoma cases were identified. One hundred seventy-five (3.9%) occurred in the head and neck, 1,934 (43.0%) occurred in the adrenal gland, and 2,391 (53.1%) occurred in “other” sites. The mean age at diagnosis was 4.21 years in the head and neck, 2.23 years in the adrenal gland, and 2.47 years in the “other” cohorts (P < .001). Two- and 5-year disease-specific survival rates were 89% and 84% in the head and neck versus 77% and 65% in the adrenal and 84% and 77% in the “other” cohorts (P < .001). The risk of disease-specific death (DSD) was higher in the adrenal cohort (adjusted hazard ratio [aHR] = 2.85; 95% CI, 1.54-5.27) compared to the head and neck cohort. Patients treated with surgery only had the lowest risk of DSD (aHR = 0.22; 95% CI, 0.13-0.35) compared to all other studied treatments. Our results demonstrate that primary neuroblastoma of the head and neck has a better prognosis than primary neuroblastoma of the adrenal gland