Description:
<jats:p> 9525 </jats:p><jats:p> Background: We previously reported in a small study (n=363) that children diagnosed with cancer who have a family history of cancer (FHC) may have worse prognosis than those without FHC, specifically children diagnosed with Hodgkin Disease (HD) and acute lymphoblastic leukemia (ALL) (Eichstadt et al., ASCO 2009). The Utah Population Database (UPDB), a unique resource, includes a Surveillance Epidemiology and End Results (SEER) registry (the Utah Cancer Registry) record-linked to extensive genealogies for 6.5 million people in which most Utah families are represented. A majority of pedigrees include ≥3 generations and several span 7+ generations. The UPDB provides an accurate, objective assessment of FHC. We expanded our original study by examining cancer mortality of 4,482 pediatric cases (age ≤18) diagnosed from 1966-2009 with adequate follow-up (median 9.5 years) and family information. Methods: We compared survival from cancer-caused mortality for pediatric cases diagnosed from 1966-2009 with FHC of any cancer diagnosed <80 yo in 1st-degree, 2nd-degree, or 3rd-degree relatives to pediatric cases without FHC. Nonparametric estimates of survivor function were determined by the life-table method and a log-rank test of homogeneity was used to test for differences between pediatric probands with FHC compared to no FHC. Results: Overall survival was significantly lower in children with positive FHC (n=1,128) than in children with no FHC (n=3,354) independent of stage (p<0.0001; 10yr survival, 69% with FHC vs.78% with no FHC). We observed lower survival in children with any leukemia and FHC (n=279) compared to children with leukemia and no FHC (n=871; p<0.0001). In particular, cases with ALL and FHC had lower survival (p<0.0001; 10yr survival, 61% vs. 75%). Pediatric lymphoma cases had lower survival when positive for FHC (n=150) vs. no FHC (n=428; p<0.0001), specifically for an HD diagnosis (p=0.0004; 10yr survival, 83% vs. 94%). Conclusions: Pediatric cancer cases with FHC of any cancer (children or adults) have decreased survival compared to cases with no FHC, specifically in children diagnosed with ALL or HD, thus validating our earlier findings. Familial factors likely contribute to increased pediatric cancer mortality. </jats:p>