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Neuroblastoma - A Neural Crest Derived Embryonal Malignancy

Journal

FRONTIERS IN MOLECULAR NEUROSCIENCE
Volume 12, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fnmol.2019.00009

Keywords

neuroblastoma; neural crest; tumorigenesis; oncogenic drivers; mutation

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Funding

  1. Swedish Childhood Cancer Fund (Barncancerfonden)
  2. Swedish Cancer Society (Cancerfonden)
  3. Swedish Foundation for Strategic Research (Stiftelsen for Strategisk Forskning)
  4. Cancer Research Foundations of Radiumhemmet (Radiumhemmets Forskningsfonder)
  5. Magnus Bergvalls stiftelse
  6. Marta and Gunnar V Philipson Foundation

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Neuroblastoma is a neural crest derived malignancy of the peripheral nervous system and is the most common and deadliest tumor of infancy. It is characterized by clinical heterogeneity with a disease spectrum ranging from spontaneous regression without any medical intervention to treatment resistant tumors with metastatic spread and poor patient survival. The events that lead to the development of neuroblastoma from the neural crest have not been fully elucidated. Here we discuss factors and processes within the neural crest that when dysregulated have the potential to be initiators or drivers of neuroblastoma development. A more precise biological understanding of neuroblastoma causes and cell of origin is highly warranted. This will give valuable information for the development of medicines that specifically target molecules within neuroblastoma cells and also give hint about the mechanisms behind treatment resistance that is frequently seen in neuroblastoma.

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