PMID- 29380702
OWN - NLM
STAT- MEDLINE
DCOM- 20180905
LR  - 20180905
IS  - 1875-6336 (Electronic)
IS  - 1573-3963 (Linking)
VI  - 14
IP  - 2
DP  - 2018
TI  - Biological and Genetic Features of Neuroblastoma and Their Clinical Importance.
PG  - 73-90
LID - 10.2174/1573396314666180129101627 [doi]
AB  - Neuroblastoma derived from primitive cells of the sympathetic nervous system 
      typically develops in the adrenal medulla or paraspinal ganglia. Neuroblastoma 
      usually occurs sporadically, but familial cases are also observed. ALK and PHOX2B 
      germline mutations can cause hereditary neuroblastoma, while a common genetic 
      variation in chromosome 6p22 is associated to sporadic neuroblastoma. However, 
      the aetiology of sporadic neuroblastoma is not exactly known. This embryonic 
      malignancy generally represents the second most common solid tumour after central 
      nervous system tumours throughout the world in childhood. Neuroblastoma is a 
      complex disease that has different clinical courses, from metastatic spread to 
      spontaneous regression. Spontaneous regression can occur without therapy in 
      primary or metastatic site. Potential regression mechanisms primarily involve 
      apoptosis, hypermethylation of subtelomeric DNA, immune response and Nerve Growth 
      Factor (NGF) deprivation. Neuroblastoma is a heterogeneous tumour that can show 
      many different chromosomal abnormalities; e.g. MYCN amplification, 1p deletion, 
      unbalanced translocations involving chromosome 17, aneuploidies and Loss of 
      Heterozygosity (LOH) events. Tyrosine kinase receptors TrkA, TrkB and TrkC, their 
      ligands NGF, Brain-derived Neurotrophic Factor (BDNF) and Neurotrophin-3 (NT-3), 
      and Aurora Kinase A (AURKA) play a regulatory role in differentiation, apoptosis, 
      cell proliferation, tumourigenesis, angiogenesis or metastasis of neuroblastoma. 
      TrkA expression is associated with differentiation or regression, depending on 
      presence or absence of NGF, whereas TrkB and BDNF are mostly expressed in 
      aggressive neuroblastomas with MYCN amplification. MYCN is amplified in 18-38% of 
      neuroblastoma cases. MYCN amplification mechanism remains to be completely 
      clarified. This paper reviews the biological/genetic features and their clinical 
      importance in neuroblastoma.
CI  - Copyright(c) Bentham Science Publishers; For any queries, please email at 
      epub@benthamscience.org.
FAU - Aygun, Nevim
AU  - Aygun N
AD  - Department of Medical Biology, Faculty of Medicine, Dokuz Eylul University, 
      Inciralti, Izmir, Turkey.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United Arab Emirates
TA  - Curr Pediatr Rev
JT  - Current pediatric reviews
JID - 101240290
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Biomarkers, Tumor/*genetics/metabolism
MH  - Child
MH  - Disease Progression
MH  - Humans
MH  - Neoplasm Metastasis
MH  - Neuroblastoma/diagnosis/*genetics/metabolism/*pathology
MH  - Prognosis
OTO - NOTNLM
OT  - Neuroblastoma
OT  - biological and genetic features
OT  - cell behaviour
OT  - cell signalling pathways
OT  - chromosomal abnormalities
OT  - clinical behaviour
OT  - clinical importance
OT  - genomic signature.
EDAT- 2018/01/31 06:00
MHDA- 2018/09/06 06:00
CRDT- 2018/01/31 06:00
PHST- 2017/08/11 00:00 [received]
PHST- 2018/01/18 00:00 [revised]
PHST- 2018/01/23 00:00 [accepted]
PHST- 2018/01/31 06:00 [pubmed]
PHST- 2018/09/06 06:00 [medline]
PHST- 2018/01/31 06:00 [entrez]
AID - CPR-EPUB-88239 [pii]
AID - 10.2174/1573396314666180129101627 [doi]
PST - ppublish
SO  - Curr Pediatr Rev. 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627.