TY  - CHAP
M1  - Book, Section
TI  - Chapter 443. Cancer Genetics and Biology
A1  - Malkin, David
A2  - Rudolph, Colin D.
A2  - Rudolph, Abraham M.
A2  - Lister, George E.
A2  - First, Lewis R.
A2  - Gershon, Anne A.
PY  - 2011
T2  - Rudolph's Pediatrics, 22e
AB  - Cancer  is  the  most  common  cause  of  disease-related  death  in  children  beyond  the  newborn  period.  The  unique  biological  features,  the  cell  of  origin,  and  the  response  to  therapy  of  childhood  cancers  make  them  intriguing  models  with  which  to  study  and  understand  the  process  of  human  carcinogenesis.  Tumors  of  childhood  commonly  reflect  the  embryonic  precursor  of  the  cell  types  in  which  they  arise.  The  majority  occur  sporadically,  and  their  etiology  remains  unclear.  Obvious  environmental  influences  to  cancer  initiation  are  not  generally  apparent.  Some  cancers,  such  as  neuroblastoma,  appear  to  arise  even  before  birth,  whereas  a  significant  number  of  others  present  with  a  striking  family  history  of  cancer  or  coincide  with  congenital  abnormalities,  suggesting  an  important  role  for  inherited  causal  genetic  factors.  Disorders  such  as  xeroderma  pigmentosum,1  which  places  children  at  increased  risk  for  developing  skin  cancers,  or  Beckwith-Wiedemann  syndrome,2  which  is  commonly  complicated  by  the  development  of  embryonal  tumors,  including  Wilms  tumor  of  the  kidney,  rhabdomyosarcoma,  or  hepatoblastoma,  are  examples  of  inherited  disorders  that  confer  an  increased  risk  developing  cancer.  Cancer  predisposition  syndromes  in  which  nonmalignant  phenotypic  features  are  not  observed  include  hereditary  retinoblastoma,3  Li-Fraumeni  syndrome,4  and  familial  polyposis,5  while  others  such  as  von  Hippel  Lindau  disease6  are  associated  with  the  coincident  presentation  of  both  benign  and  malignant  neoplasms.  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=7041362
ER  -