« ‘Robbie and Coron Pathologic Bess sense Kirn Abus, Aster (Chere 20150) POF e (Chapter 5). The chromosomal regan implicated if BWS has been localized to band 11p15.5 (" WT2"), distal to the IYTT locus, This region contains multiple genes that are normally expressed from only one of the two parental alleles, with transcriptional silencing (Ge, imprinting) of the other parental homologue by methylation of the promoter region. Unlike WAGE or Denys-Drash syndromes, the genetic basis for BWS is considerably more heterogeneous in that no single Upl55 gene is involved in all cases. Moreover, the phenotype of BWS, including the predisposition to tumorigenesis, is influenced by the specific “WT2" imprinting abnormalities present. One of the genes in Grosely, Wits tumor tonds to present as a large, softy, wol- Corcumserbed mass, athough 10% are ater lateral or mul ‘conti at the time of ciagnosis..On cut section, the tumor is Soft, Homagensous, and tan to gray wth occasional foc of hemiorage, eyst formation, and necrosis i. 12-28) Microscopically, Wiis tumors are characterized by recogriz~ _ablaatlempts to recaptuate dfirent staves of neptrogenast mon. Farsly, afer haoroogeus anon ate. cenifod, ineuseg samous & mucness eptaium. srocth muse, aspose tase, carage, and sted aed neurogenic Hs Approxmately 5% of tumors reveal anaplasia, detined as the receice of cole with ago, hyperctremate, pleomerphic uel aod abnor mtoaes (i. 10-228), The presence of anaclasia crates wt the presence of TPS ruitions and tho emorganes of reitans to onemotherery, Real tat pS @licits pro-apoptotic signals in response to DNA damage