RT Book, Section
A1 Brodeur, Garrett M.
A2 Valle, David L.
A2 Antonarakis, Stylianos
A2 Ballabio, Andrea
A2 Beaudet, Arthur L.
A2 Mitchell, Grant A.
SR Print(0)
ID 1181418248
T1 Clinical and Biological Aspects of Neuroblastoma
T2 The Online Metabolic and Molecular Bases of Inherited Disease
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9780071459969
LK ommbid.mhmedical.com/content.aspx?aid=1181418248
RD 2024/04/19
AB Neuroblastoma,  a  tumor  of  the  postganglionic  sympathetic  nervous  system,  is  the  most  common  extracranial  solid  tumor  of  childhood.No  environmental  exposures  or  agents  have  been  associated  with  an  increased  risk  of  neuroblastoma.  However,  a  subset  has  a  genetic  predisposition  that  follows  an  autosomal  dominant  pattern  of  inheritance.Primary  tumors  generally  arise  in  the  adrenal  medulla  (50  percent)  or  elsewhere  in  the  abdomen  or  pelvis  (30  percent).  Only  20  percent  arise  in  the  chest.Metastases  usually  are  found  in  the  regional  lymph  nodes,  bone,  bone  marrow,  skin,  or  liver.  Paraneoplastic  syndromes  characteristic  of  neuroblastomas  are  seen  in  a  small  percentage  of  patients.Tissue  biopsy  or  characterization  of  cells  in  the  bone  marrow  diagnoses  neuroblastomas.  Catecholamine  metabolites  are  elevated  in  the  urine  in  over  90  percent  of  cases.There  is  an  international  neuroblastoma  staging  system  for  categorizing  the  extent  and  resectability  of  the  primary  tumor  and  the  presence  or  absence  of  metastases.A  number  of  prognostic  variables  have  been  identified  that  allow  better  prediction  of  clinical  behavior:  MYCN  amplification,  allelic  loss  of  1p36,  gain  of  17q,  expression  of  TrkA,  tumor  cell  ploidy,  and  tumor  pathology.Histologically,  neuroblastic  tumors  can  be  immature  (neuroblastoma),  partially  mature  (ganglioneuroblastoma),  or  completely  mature  (ganglioneuroma).  However,  other  features,  such  as  the  presence  or  absence  of  Schwannian  stroma,  mitoses,  or  karyorrhectic  cells,  may  have  more  prognostic  importance.Localized,  resectable  tumors  can  be  cured  by  surgery  alone.  Unresectable  tumors  or  metastatic  disease  in  infants  require  mild  to  moderately  intensive  chemotherapy.  Metastatic  disease  in  older  patients,  and  regional  tumors  with  unfavorable  biological  features  in  any  age,  require  intensive  chemotherapy,  frequently  with  bone  marrow  or  stem  cell  rescue.Screening  of  infants  for  neuroblastoma  by  measuring  urinary  catecholamine  metabolites  has  resulted  in  a  doubling  of  the  apparent  incidence  rate  in  infants  with  no  decrease  in  advanced  disease  in  older  children.Future  therapeutic  approaches  may  be  aimed  at  the  induction  of  differentiation  or  programmed  cell  death  through  retinoid  or  neurotrophin  receptor  pathways.  Alternate  approaches  may  focus  on  the  product  of  the  MYCN  gene,  the  1p36  suppressor  gene,  other  specific  genetic  changes,  or  antiangiogenesis.