XXXX in adults: Incidence and survival analysis based on SEER data.

@entity1 @entity5 @entity521
PURPOSE: Clinical data on survival outcomes of adult @entity1 (defined as 20 years of age and older) with @entity521 are scarce due to the rarity of the disease. Small single institution reports have described worse outcomes for adults than pediatric @entity1 . 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 @entity521 . Analyses compared frequency and survival rates of @entity521 among @entity1 and adults with particular focus on the incidence, case characteristics, and long-term survival of the adult @entity1 . RESULTS: The observed 3- and 5-year survival rates were lowest among adult @entity1 (45.9% and 36.3%, respectively), whereas @entity1 fared best with 86.0% 3-year and 84.6% 5-year overall survival. There was a steady decline in the incidence of @entity521 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 @entity521 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 @entity521 have significantly worse outcome than @entity1 . This may be due to @entity5 biology, more virulent clinical course, or possibly due to the fact that adults are less sensitive or have poor tolerance to pediatric chemotherapy regimens.
