RT Book, Section
A1 Cuglievan, Branko
A1 Zaky, Wafik
A1 Gorlick, Richard
A1 Harrison, Douglas
A2 Kantarjian, Hagop M.
A2 Wolff, Robert A.
A2 Rieber, Alyssa G.
SR Print(0)
ID 1190838981
T1 Pediatric Cancers
T2 The MD Anderson Manual of Medical Oncology, 4e
YR 2022
FD 2022
PB McGraw Hill Education
PP New York, NY
SN 9781260467642
LK hemonc.mhmedical.com/content.aspx?aid=1190838981
RD 2024/04/17
AB KEY  CONCEPTSPatients  with  many  common  pediatric  neoplasms,  such  as  acute  lymphoblastic  leukemia  (ALL),  Wilms  tumor,  non-Hodgkin  lymphoma,  Hodgkin  lymphoma,  and  germ  cell  tumors,  have  survival  rates  approaching  or  even  exceeding  90%.  However,  progress  has  been  limited  for  patients  with  some  types  of  solid  tumors,  high-grade  gliomas,  and  diffuse  intrinsic  brainstem  gliomas.Patients  with  ALL,  the  most  common  cancer  in  children,  typically  present  with  hepatomegaly  or  splenomegaly,  pallor,  fever,  and/or  bruising.  ALL's  genetic  interrogation  has  enabled  us  to  employ  risk-based  treatment  strategies  that  have  led  to  a  cure  rate  greater  than  98%  in  some  ALL  subtypes.  Those  with  unfavorable  cytogenetic  factors,  suboptimal  responses  to  chemotherapy,  or  those  who  relapse  require  treatment  with  aggressive  regimens  and  will  benefit  from  novel  treatment  modalities.Hodgkin  lymphoma,  the  most  common  neoplasm  among  adolescents  ages  15  to  19,  commonly  presents  with  painless  supraclavicular  or  cervical  adenopathy  with  or  without  the  involvement  of  the  mediastinum.  Fever  higher  than  38  °C,  drenching  night  sweats,  and  weight  loss  more  than  10%  of  body  weight  within  6  months  have  been  classified  as  “B  symptoms”  and  affect  staging  and  prognosis.  Interim  assessment  with  18-fluorodeoxyglucose–positron  emission  tomography  scan  has  allowed  recognizing  favorable  responders  who  benefit  from  less  toxic  therapy.In  children,  the  central  nervous  system  (CNS)  neoplasms  are  mostly  infratentorial.  Patients  present  with  increased  intracranial  pressure,  which  manifests  as  macrocephaly,  vomiting,  and  developmental  delay  in  infants  and  headache,  vomiting,  blurred  vision,  and  altered  mental  status  in  older  children.  A  gross  total  resection  is  the  surgery  goal,  whenever  it  is  safe  and  feasible.  Chemotherapy  and  radiation  therapy  typically  follow  for  the  malignant  CNS  tumors  except  for  chemoresistant  tumors  like  ependymoma.Non-neural  solid  tumors  vary  considerably  by  histology,  tumor  cell  of  origin,  symptom  spectrum,  age  at  presentation,  treatment,  and  outcome.  The  majority  are  treated  with  a  multidisciplinary  approach  that  includes  surgical  resection,  radiation,  and/or  systemic  chemotherapy.  In  some  instances,  like  neuroblastoma  and  Wilms  tumor,  treatment  is  also  determined  by  the  patient's  risk  status,  which  incorporates  biologic  features.In  pediatric  cancer  survivors,  long-term  side  effects  of  therapy  include  cardiovascular  dysfunction  in  those  exposed  to  anthracyclines  and/or  radiation,  neurocognitive  deficits,  endocrinopathies,  and  secondary  CNS  tumors  in  patients  who  received  radiation  to  the  CNS,  and  breast  and  thyroid  cancer  for  those  exposed  to  radiation  to  the  chest.