443 paediatric cases of XXXX registered with the German Central @entity320 Registry between 1983 and 2011.

@entity1 @entity5 @entity1066 @entity320 @entity1349
BACKGROUND: @entity320 is a very rare paediatric @entity5 . This study was performed in order to understand clinical features and prognosis of @entity320 in @entity1 and adolescents. METHODS: 443 @entity1    18years of age with @entity320 were prospectively registered with the German Central @entity320 Registry between 1983 and 2011. Cases were collected from 58 participating centres. 276 paediatric cases with a follow-up >3months were evaluated for survival probabilities and prognostic factors by Kaplan-Meier method. RESULTS: Age of diagnosis ranged from 3months to 18years (median age 16years). The male to female ratio was 0.8 (202 male, 240 female). Most @entity320 were located at the trunk (n=195) and the lower extremity (n=114). @entity1 with >3months of follow-up (median 55months) showed an @entity1349 ( @entity1349 ) of 94.8% in 5years. Survival according to @entity5 stage was 98.5% for stage I (n=190), 91.1% for stage II (n=39) and 53.0% for stage III/IV @entity5 (n=11). Worse outcome was seen in @entity1 with @entity320 ( @entity1349 77.9%, n=42) compared to superficial spread histotype ( @entity1349 100%, n=138) or other histotype ( @entity1349 96.9%, n=88) (p<0.0001), in case of thicker @entity5 (Clark level IV or V, @entity1349 87.1%, n=84) compared to thinner @entity5 (Clark level I, II, III, @entity1349 99.1%, n=164) (p=0.0008) and in case of @entity1066 ( @entity1349 65.6%, n=17) compared to no @entity1066 ( @entity1349 99.2%, n=182). CONCLUSION: @entity1 and @entity5 characteristics in paediatric @entity320 @entity1 show no evident differences to adult @entity320 cases. The same clinical approach as in adults should be used.
