Type:
Educational Exhibit
Keywords:
Prostheses, Neoplasia, Cancer, Drainage, Diagnostic procedure, Biopsy, MR-Cholangiography, MR, CT, Oncology, Biliary Tract / Gallbladder, Abdomen
Authors:
S. Mghaieth, O. Ghdes, N. Achour, B. Fraj, N. Mnif; Tunis/TN
DOI:
10.26044/ecr2019/C-3632
Background
Cholangiocarcinoma (CC) is a malignant primary tumor that originates in the epithelium of the biliary ducts,
and that poses a diagnostic and therapeutic challenge owing to the broad range of histologic types,
growth patterns,
and clinical manifestations,
along with varying imaging manifestations that can overlap those of other hepatobiliary diseases.
It is the second most frequent hepatobiliary tumor after hepatocellular carcinoma,
diagnosed usually in patients above 65 years of age with a slight prevalence in men,
and is predominantly adenocarcinoma (95% of cases) at histologic analysis.
Its etiology is unknown and most cases occur sporadically; but there are several well-defined risk factors,
the most common of these is primary sclerosing cholangitis.
A mutation in the p53 tumor suppressor gene and k-ras gene has been demonstrated in peripheral and extrahepatic cholangiocarcinomas respectively,
a finding that suggests a genetic link.
Surgical resection of the tumor remains the only potentially curable therapy,
leading to a 5-year survival of 30 to35%.
However,
most patients present late with an unresectable tumor.
Treatment options in advanced disease are limited and the prognosis is poor,
with dismal 5-year disease-free survival rates.