KEGG   DISEASE: NeuroblastomaHelp
Entry
H00043                      Disease                                

Name
Neuroblastoma
Description
Neuroblastoma is a tumor derived from primitive cells of the sympathetic nervous system and is the most common solid tumor in childhood. Approximately one-half of children have localized tumors that can be cured with surgery alone. These favorable tumors are characterized by near-triploid karyotypes with whole chromosome gains. These tumors rarely have structural rearrangements, and they usually express the TrkA neurotrophin receptor. Patients with these tumors are more likely to be less than 1 year of age. The remaining children have widespread metastatic disease or quite large, aggressive, localized tumors. These unfavorable tumors are characterized by structural changes, including deletions of 1p or 11q, unbalanced gain of 17q and/or amplification of the MYCN protooncogene. They might also express the TrkB neurotrophin receptor and its ligand, brain-derived neurotrophic factor (BDNF). These patients are usually older than 1 year of age, and have a poor long-term survival rate of approximately 30%.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of eye, brain, and central nervous system
   H00043  Neuroblastoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of brain or central nervous system
   2A02  Primary neoplasm of spinal cord, cranial nerves or remaining parts of central nervous system
    H00043  Neuroblastoma
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of middle ear, respiratory or intrathoracic organs
     2C20  Malignant neoplasms of nasal cavity
      H00043  Neuroblastoma
Tumor markers [br08442.html]
 H00043
Cancer-accociated carbohydrates [br08441.html]
 H00043
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00131  Amplified MYCN to transcriptional activation
N00132  Amplified MYCN to transcriptional repression
Gene
MYCN (normal/amplified) [HSA:4613] [KO:K09109]
TrkA (high/low expression) [HSA:4914] [KO:K03176]
TrkB (low/high expression) [HSA:4915] [KO:K04360]
TrkC (high/low expression) [HSA:4916] [KO:K05101]
Drug
Cyclophosphamide [DR:D00287]
Doxorubicin hydrochloride [DR:D01275]
Comment
ICD-O: 9500/3, Tumor type: Neuroblastoma
Other DBs
ICD-11: 2A02.1 2C20.3
ICD-10: C38 C47 C48 C72 C74 C76
MeSH: D009447
Reference
PMID:12612655 (gene, tumor type)
  Authors
Brodeur GM.
  Title
Neuroblastoma: biological insights into a clinical enigma.
  Journal
Nat Rev Cancer 3:203-16 (2003)
DOI:10.1038/nrc1014
Reference
  Authors
van Noesel MM, Versteeg R.
  Title
Pediatric neuroblastomas: genetic and epigenetic 'danse macabre'.
  Journal
Gene 325:1-15 (2004)
DOI:10.1016/j.gene.2003.09.042

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