Primary Intrahepatic Biliary Tracts Tumors: Diagnosis and Treatment

The most frequent primary intrabiliary tracts tumors are intrabiliary cholangiocarcinoma and cystadenocarcinoma. Cholangiocarcinoma is a primary adenocarcinoma developed from the bile ducts cells and represents 15% of primary liver cancers. The cystadenocarcinoma often corresponds to the transformation of a biliary cystadenoma, which can develop on congenital anomalies of the bile ducts such as Caroli's disease, congenital hepatic fibrosis, hepatic polycystosis or bile duct cysts. The clinical symptoms are nonspecific and these primary intrabiliary tracts tumors are discovered at advanced stage. The upper right quadrant pain with abdominal mass are the typical signs. Their management depends on the stage and location of the tumor at the diagnosis time. The treatment is therefore surgical. The resectability rate of intrahepatic cholangiocarcinomas is between 50 and 70%. No systemic chemotherapy has yet proven its effectiveness in the treatment of cholangiocarcinoma. The prognosis for cholangiocarcinoma is poor. Early detection of intrahepatic cholangiocarcinoma is difficult, and the median survival at diagnosis is 12 months in the absence of surgical treatment. The prognosis of cystadecarcinoma is also poor but remain better than that of cholangiocarcinoma.