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%.
Human diseases [BR:
Cancers of eye, brain, and central nervous system
Human diseases in ICD-11 classification [BR:
Neoplasms of brain or central nervous system
2A02 Primary neoplasm of spinal cord, cranial nerves or remaining parts of central nervous system
Malignant neoplasms, except primary neoplasms 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
Tumor markers [
Cancer-associated carbohydrates [
Pathways in cancer
Transcriptional misregulation in cancer
Neurotrophin signaling pathway
Amplified MYCN to transcriptional activation
Amplified MYCN to transcriptional repression
MYCN (normal/amplified) [HSA:
TrkA (high/low expression) [HSA:
TrkB (low/high expression) [HSA:
TrkC (high/low expression) [HSA:
(NBLST1) KIF1B [HSA:
(NBLST2) PHOX2B [HSA:
(NBLST3) ALK [HSA:
Vincristine sulfate [DR:
Doxorubicin hydrochloride [DR:
ICD-O: 9500/3, Tumor type: Neuroblastoma
C38 C47 C48 C72 C74 C76
(gene, tumor type)
Neuroblastoma: biological insights into a clinical enigma.
Nat Rev Cancer 3:203-16 (2003)
van Noesel MM, Versteeg R.
Pediatric neuroblastomas: genetic and epigenetic 'danse macabre'.
Gene 325:1-15 (2004)
Schlisio S, Kenchappa RS, Vredeveld LC, George RE, Stewart R, Greulich H, Shahriari K, Nguyen NV, Pigny P, Dahia PL, Pomeroy SL, Maris JM, Look AT, Meyerson M, Peeper DS, Carter BD, Kaelin WG Jr
The kinesin KIF1Bbeta acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressor.
Genes Dev 22:884-93 (2008)
Trochet D, Bourdeaut F, Janoueix-Lerosey I, Deville A, de Pontual L, Schleiermacher G, Coze C, Philip N, Frebourg T, Munnich A, Lyonnet S, Delattre O, Amiel J
Germline mutations of the paired-like homeobox 2B (PHOX2B) gene in neuroblastoma.
Am J Hum Genet 74:761-4 (2004)
Mosse YP, Laudenslager M, Longo L, Cole KA, Wood A, Attiyeh EF, Laquaglia MJ, Sennett R, Lynch JE, Perri P, Laureys G, Speleman F, Kim C, Hou C, Hakonarson H, Torkamani A, Schork NJ, Brodeur GM, Tonini GP, Rappaport E, Devoto M, Maris JM
Identification of ALK as a major familial neuroblastoma predisposition gene.
Nature 455:930-5 (2008)
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