Heterogeneous cytogenetic subgroups and outcomes in childhood XXXX : a retrospective international study.

@entity1 @entity11626 @entity2592 @entity3052 @entity1017 @entity10706 @entity1349
UNASSIGNED: Comprehensive clinical studies of @entity1 with @entity10706 ( @entity10706 ) are lacking. We performed an international retrospective study on 490 @entity1 (age  <=18 years) with non-Down syndrome de novo @entity10706 diagnosed from 1989 to 2009. @entity1 with @entity10706 (median age 1.53 years) comprised 7.8% of @entity3052 . Five-year event-free (EFS) and @entity1349 ( @entity1349 ) were 43.7%  2.7% and 49.0%  2.7%, respectively. @entity1 diagnosed in 2000-2009 were treated with higher doses of @entity2592 and had better EFS (P=0.037) and @entity1349 (P=0.003) than those diagnosed in 1989-1999, but outcomes of @entity1 diagnosed in 2000-2004 and 2005-2009 were similar. Transplantation in first remission did not improve survival. Cytogenetic data were available for 372 (75.9%) @entity1 : hypodiploid (n=18, 4.8%), normal karyotype (n=49, 13.2%), pseudodiploid (n=119, 32.0%), 47-50 chromosomes (n=142, 38.2%), and >50 chromosomes (n=44, 11.8%). @entity1017 occurred in 195 of 372 (52.4%) @entity1 : +21 (n=106, 28.5%), +19 (n=93, 25.0%), +8 (n=77, 20.7%). Losses occurred in 65 @entity1 (17.5%): -7 (n=13, 3.5%). Common structural chromosomal aberrations were t(1;22)(p13;q13) (n=51, 13.7%) and 11q23 rearrangements (n=38, 10.2%); t(9;11)(p22;q23) occurred in 21 @entity1 . On the basis of frequency and analysis for EFS and @entity1349 , @entity10706 can be classified to 3 risk groups: good risk: 7p abnormalities; poor risk: normal karyotypes, -7, 9p abnormalities including t(9;11)(p22;q23)/ @entity11626 -MLLT3, -13/13q-, and -15; and intermediate risk: others including t(1;22)(p13;q13)/OTT-MAL and 11q23/ @entity11626 except t(9;11). Risk-based innovative therapy is needed to improve @entity1 outcomes.
