Genetics of Beckwith-Wiedemann syndrome-associated tumors: Common genetic pathways

A specific subset of solid childhood tumors-Wilms' tumor, adrenocortical ca rcinoma, rhabdomyosarcoma, and hepatoblastoma-is characterized by its assoc iation with Beckwith-Wiedemann syndrome. Genetic abnormalities found in the se tumors affect the same chromosome region (11p15), which has been implica ted in the etiology of Beckwith-Wiedemann syndrome. This suggests that the development of these tumors occurs along a common genetic pathway involving chromosome 11. To search for additional common genetic pathways, this arti cle reviews the genetic data published for these tumors. It was found that, up until now, the only genetic abnormalities detected in all four tumors a ffect chromosome band 11p15 and the TP53 gene. In addition, there are sever al aberrations that occur in two or three of the neoplasms. It is concluded that, of the four tumors, the genetic relationship is most evident between Wilms' tumor and rhabdomyosarcoma. Genes Chromosomes Cancer 28:1-13, 2000. (C) 2000 Wiley-Liss, Inc.