PMID- 17431781
OWN - NLM
STAT- MEDLINE
DCOM- 20071113
LR  - 20220408
IS  - 0163-2116 (Print)
IS  - 0163-2116 (Linking)
VI  - 52
IP  - 11
DP  - 2007 Nov
TI  - Incidence, diagnosis, and therapy of cholangiocarcinoma in patients with primary 
      sclerosing cholangitis.
PG  - 3123-35
AB  - Primary sclerosing cholangitis (PSC) can lead to the development of 
      cholangiocarcinoma (CCA). The tumor may present as an intrahepatic focal 
      cholangiocellular carcinoma but more often as a ductal infiltrating desmoplastic 
      lesion. CCA is found synchronously with the diagnosis of PSC in 20-30% and within 
      1 year in 50%. During later follow-up, the yearly developmental rate of CCA is 
      0.5-1.5%. Most patients with PSC and CCA do not yet have cirrhosis but present 
      with a severe stenosis at the hilum of the liver. This type of tumor is difficult 
      to diagnose by imaging techniques.(18)F-FDG-PET scanning and CEA or CA 19-9 are 
      not early diagnostic tools. Regular MRI, multislice CT, and repeated 
      endoscopically obtained brush cytology of stenotic lesions are recommended. The 
      recent use of more extensive surgical resection techniques in patients with CCA 
      results in 5-year survival rates of > or =50%. If tumors are small or incidental 
      findings, liver transplantation leads to a 3- to 5-year survival rate of 35%. 
      Pretransplant radiotherapy with 5-FU chemosensitization followed by endoscopic 
      brachytherapy with iridium-192 seems to greatly improve the outcome of 
      transplantation. Treatment with ursodeoxycholic acid may prevent development of 
      CCA.
FAU - Fevery, Johan
AU  - Fevery J
AD  - Division of Hepatobiliary, University Hospital Gasthuisberg, Catholic University 
      of Leuven, B3000 Leuven, Belgium. Johan.Fevery@med.kuleuven.be
FAU - Verslype, Chris
AU  - Verslype C
FAU - Lai, Gillian
AU  - Lai G
FAU - Aerts, Raymond
AU  - Aerts R
FAU - Van Steenbergen, Werner
AU  - Van Steenbergen W
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20070412
PL  - United States
TA  - Dig Dis Sci
JT  - Digestive diseases and sciences
JID - 7902782
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
MH  - Belgium/epidemiology
MH  - Bile Duct Neoplasms/diagnosis/*epidemiology/etiology/therapy
MH  - *Bile Ducts, Intrahepatic
MH  - Biopsy
MH  - Brachytherapy/methods
MH  - Cholangiocarcinoma/diagnosis/*epidemiology/etiology/therapy
MH  - Cholangiopancreatography, Endoscopic Retrograde
MH  - Cholangitis, Sclerosing/*complications
MH  - Diagnosis, Differential
MH  - Disease Progression
MH  - Endosonography
MH  - Fatal Outcome
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Incidence
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Positron-Emission Tomography
MH  - Retrospective Studies
MH  - Tomography, X-Ray Computed
EDAT- 2007/04/14 09:00
MHDA- 2007/11/14 09:00
CRDT- 2007/04/14 09:00
PHST- 2005/12/20 00:00 [received]
PHST- 2006/11/08 00:00 [accepted]
PHST- 2007/04/14 09:00 [pubmed]
PHST- 2007/11/14 09:00 [medline]
PHST- 2007/04/14 09:00 [entrez]
AID - 10.1007/s10620-006-9681-4 [doi]
PST - ppublish
SO  - Dig Dis Sci. 2007 Nov;52(11):3123-35. doi: 10.1007/s10620-006-9681-4. Epub 2007 
      Apr 12.