The clinical phenotype of @entity35795 - XXXX /E positive pediatric @entity8 .

@entity1 @entity8 @entity35795 @entity5 @entity406 @entity40992
UNASSIGNED: Clear cell @entity8 (CCSK) although uncommon, is the second most frequent @entity8 of childhood. Until now, the sole recurrent genetic aberration identified in CCSKs is t(10;17)(q22;p13), which gives rise to a fusion transcript of @entity35795 and @entity40992 /E. So far, the clinical relevance of this fusion transcript is unknown. The aim of this descriptive study was to determine the clinical phenotype of t(10;17)(q22;p13) positive CCSKs. Snap-frozen tissues, @entity406 -fixed paraffin-embedded tissues or RNA previously extracted from CCSK samples throughout European, North-American and Japanese study groups were screened by RT-PCR for the @entity35795 - @entity40992 /E transcript. Clinical characteristics, @entity5 characteristics, and outcome of @entity1 with and without the fusion transcript were studied. The cohort comprised 51 previously published cases to which were added 139 internationally collected CCSK samples. RNA from 57 of these additionally collected cases was of sufficient quality to be successfully screened for the @entity35795 - @entity40992 /E transcript. In total, seven of the 108 cases harbored the fusion transcript. @entity1 with @entity5 containing the fusion transcript were relatively young (median age 10 months), had associated low median @entity5 volumes and stage I disease was not observed in these @entity1 . Two of seven @entity1 relapsed and one of seven @entity1 died of disease. Ranges of values were not overtly different between @entity1 with and without the fusion transcript; however, the number of fusion transcript positive cases turned out to be too small to permit reliable statistical analysis. The current study did not identify an explicit clinical phenotype of CCSK cases harboring the @entity35795 - @entity40992 /E fusion transcript.    2015 Wiley Periodicals, Inc.
