RT Book, Section
A1 Makawita, Shalini
A1 Lee, Sunyoung
A1 Chun, Yun Shin
A1 Roach, Millicent A.
A1 Koay, Eugene J.
A1 Javle, Milind
A2 Kantarjian, Hagop M.
A2 Wolff, Robert A.
A2 Rieber, Alyssa G.
SR Print(0)
ID 1190835403
T1 Biliary Tract Cancer
T2 The MD Anderson Manual of Medical Oncology, 4e
YR 2022
FD 2022
PB McGraw Hill Education
PP New York, NY
SN 9781260467642
LK accessmedicine.mhmedical.com/content.aspx?aid=1190835403
RD 2024/04/19
AB KEY  CONCEPTSBiliary  tract  cancers  are  rare  tumors  (prevalence  <6/100,000  persons/year)  with  significant  geographic  variability.Risk  factors  include  cholelithiasis,  polyps,  porcelain  gallbladder,  chronic  inflammatory  states  such  as  inflammatory  bowel  disease,  primary  sclerosing  cholangitis  and  nonalcoholic  steatohepatitis,  genetic  syndromes  (Peutz-Jeghers  or  Gardner  syndrome),  and  chronic  infection  (Salmonella  typhi  carriers,  Opisthorchis  viverrine,  and  Clonorchis  sinensis,  particularly  in  Southeast  Asia,  hepatitis  B  and  C).Five-year  overall  survival  rates  post  R0  resection  range  from  40%  to  50%.  High-risk  features  include  positive  lymph  nodes/margins,  vascular  invasion,  and  multifocal  disease.In  localized  disease,  a  multimodality  approach  is  often  used:In  early-stage  intrahepatic  cholangiocarcinoma,  surgical  resection  with  nodal  dissection  is  followed  by  adjuvant  capecitabine  or  gemcitabine-cisplatin;  radiation  therapy  is  given  postoperatively  for  positive  margins.Neoadjuvant  chemotherapy  is  considered  for  high-risk  features.In  clinical  practice,  combination  chemotherapy  (gemcitabine-cisplatin  or  gemcitabine-capecitabine)  is  used  adjuvantly,  particularly  in  node-positive  disease  or  R1  resection.Systemic  therapy  in  metastatic  disease  is  guided  largely  by  molecular  profiling  and  actionable  targets.  FGFR,  IDH1,  HER2,  and  BRAF  alterations  are  among  the  most  enriched,  and  numerous  clinical  trial  options  are  available  for  these  patients.Cholangiocarcinoma  has  low  rates  of  high  microsatellite  instability  and  programmed  cell  death  ligand  1  positivity.  It  remains  overall  a  “cold  tumor,”  though  methods  to  improve  immune  cell  infiltration  and  predict  responders  to  immunotherapy  are  underway.