title: New insights into the genetics of neuroblastoma.
abstract: Neuroblastoma is a genetically and clinically heterogeneous tumor of childhood, arising from precursor cells of the sympathetic nervous system. It is still a challenging cancer for pediatric oncology, as some tumors will spontaneously regress, while others will become refractory to all forms of therapy. The clinical course of this disease is greatly influenced by both patient age and the genetic abnormalities that occur within the tumors. MYCN (v-myc myelocytomatosis viral related oncogene, neuroblastoma derived (avian)) amplification and loss of chromosome 11q heterozygosity have been known to be indicative of poor prognosis. In this article, we review how mutations and structural alterations in specific genes contribute to inheritable predisposition to neuroblastoma and/or to aggressive disease pathogenesis, as well as implications for diagnosis and therapy. These genes include PHOX2B (paired-like homeobox 2b), ALK (anaplastic lymphoma receptor tyrosine kinase), and ATRX (alpha thalassemia/mental retardation syndrome X-linked).
authors: School of Medicine, Royal College of Surgeons in Ireland, Bahrain, Manama, Bahrain.
authors: Srishma
authors: S
authors: Sridhar
authors: Batool
authors: B
authors: Al-Moallem
authors: Hawra
authors: H
authors: Kamal
authors: Marta
authors: M
authors: Terrile
authors: Raymond L
authors: RL
authors: Stallings
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D000818:Animals; D004265:DNA Helicases / Q000235:genetics; D020022:Genetic Predisposition to Disease / Q000235:genetics; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D019656:Loss of Heterozygosity / Q000235:genetics; D000071447:N-Myc Proto-Oncogene Protein; D009447:Neuroblastoma / Q000235:genetics*; D009687:Nuclear Proteins / Q000235:genetics; D015513:Oncogene Proteins / Q000235:genetics; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D014157:Transcription Factors / Q000235:genetics; D000075924:X-linked Nuclear Protein
chemical_list: D018398:Homeodomain Proteins; C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; C109220:NBPhox protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins; D014157:Transcription Factors; C000626173:ALK protein, human; C000626174:Alk protein, mouse; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases; D004265:DNA Helicases; C490462:Atrx protein, mouse; D000075924:X-linked Nuclear Protein