MYCN-mediated murine cancer models

High expression of MYCN can facilitate tumorigenic processes in cancer entities of both, infants and adults. Elevated MYCN levels are often driven by amplification of the MYCN-oncogene and MYCN activity is linked to poor clinical outcome in a variety of cancer types [1]. Among solid tumors of childhood, MYCN amplification is prominently observed in neuroblastoma and medulloblastoma, whereas MYCN copy number alterations are found in several common tumors of adulthood, including small cell lung cancer (Fig. 1). Although the importance of MYCN in cancer development and maintenance of an aggressive phenotype has been widely recognized for decades, MYCN had been considered a poor drug target. With the recent advent of small molecule inhibitors indirectly interfering with MYCN functions, it now becomes essential to establish MYCN-driven in vivo tumor models that recapitulate human disease types. For this purpose, we and others have characterized genetically engineered mouse strains that use MYCN upregulation to specifically induce tumor formation. In two murine models of MYCN-driven neuroblastoma, TH-MYCN [2] and LSL-MYCN;Dbh-iCre [3], MYCN overexpression in neural crest cells resulted in tumors closely resembling human neuroblastoma in terms of tumor localization and histology, genomic aberrations Editorial