Neuroblastoma in adults: Incidence and survival analysis based on SEER data

Abstract

Purpose: Clinical data on survival outcomes of adult patients (defined as 20 years of age and older) with neuroblastoma are scarce due to the rarity of the disease. Small single institution reports have described worse outcomes for adults than pediatric patients.

Materials/methods: Data from the public access Surveillance Epidemiology and End Results database were reviewed for a 30-year period between 1973 and 2002 for the diagnosis of neuroblastoma. Analyses compared frequency and survival rates of neuroblastoma among children and adults with particular focus on the incidence, case characteristics, and long-term survival of the adult patients.

Results: The observed 3- and 5-year survival rates were lowest among adult patients (45.9% and 36.3%, respectively), whereas infants fared best with 86.0% 3-year and 84.6% 5-year overall survival. There was a steady decline in the incidence of adult neuroblastoma from 0.47 cases per million per year in 1973-1977 to 0.12 cases per million per year in 1998-2002. When the long-term adult neuroblastoma survival was examined in detail for 1-, 3-, 5-, 7-, and 10-year follow-up periods, there was continuous decrease in survival during the first 7 years after the diagnosis with subsequent stabilization for both observed (OS) and relative survival (RS) rates. There was a notable trend towards improved outcome over the past three decades.

Conclusions: Adults with neuroblastoma have significantly worse outcome than children. This may be due to tumor biology, more virulent clinical course, or possibly due to the fact that adults are less sensitive or have poor tolerance to pediatric chemotherapy regimens.