Incidence, Diagnosis, and Therapy of Cholangiocarcinoma in Patients with Primary Sclerosing Cholangitis

Abstract

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.

Endomicroscopy in bile duct: Inflammation interferes with pCLE applied in the bile duct: A prospective study of 54 patients

“…Cholangiocarcinoma occurs in the hilar region in about 65% of cases, in the distal common bile duct in 20%, and as an intrahepatic lesion in 15%. 1,2 Surgical resection of Klatskin tumours comprises extrahepatic suprapancreatic bile duct resection and hepatic resection combined with resection of the portal vein and caudate lobe, and lymph node dissection. In this approach, mortality rates have been reported to range from 7.5 to 18% and morbidity rates from 19 to 85%.…”

Liver transplantation and whipple surgery combined with chemoradiotherapy for treatment of hilar cholangiocarcinoma in patients with primary sclerosing cholangitis

“…5 ERCP with brush cytology and tumor markers has been applied to PSC patients for the detection of CC with limited success. 6 Therefore, the entire biliary epithelium is at risk of a malignant transformation.…”

Cholangiocarcinoma complicating recurrent primary sclerosing cholangitis after liver transplantation

“…Occult cholangiocarcinoma is found in 3% of explanted PSC livers at transplant. It has been estimated that the annual incidence of cholangiocarcinoma in PSC is 1-1.5% and is a leading cause of death [11]. The risk factors for developing cholangiocarcinoma in PSC are not established, smoking and alcohol have been suggested but duration of PSC has not been found to be associated [12].…”

“…The mean time from PSC diagnosis to death or transplantation ranges from 9.6 to 12 years, with cholangiocarcinoma developing in [8][9][10][11][12][13].2% of patients [3,4]. Indications for transplantation include complications of portal hypertension, impaired quality of life and chronic liver failure.…”