Introduction:

Hilar cholangiocarcinoma is a rare tumor, with poor prognosis and high mortality, with a silent course until the advanced stage of the disease.

Objective:

To describe the etiopathogenesis and imaging diagnosis of hilar cholangiocarcinoma.

Methods:

From the PubMed, SciELO and Latindex databases, articles published from 2005 to May 2020 were selected, insofar they were related to hilar/perihilar cholangiocarcinoma: etiopathogenesis, clinical diagnosis, laboratory studies and imaging studies.

Development:

Obstructive jaundice (90%), weight loss (60-75%) and abdominal pain (40%) are the most frequent presenting symptoms. Predisposing factors, either genetic and environmental, trigger chronic inflammatory responses that damage the DNA of ductal cells, causing abnormal cell differentiation with the development of cholangiocarcinoma. Parasitic infections and bile duct stone disease in Eastern countries and primary sclerosing cholangitis in the West are the main predisposing factors. The tripod for diagnosis is made up of ultrasound, computerized axial tomography and nuclear magnetic resonance.

Conclusions:

Hilar cholangiocarcinoma is a rare cause of cholestasis among the general population, with a predominance during the sixth decade of life and among males, jaundice being the reason for consultation of these patients, in which ultrasound and computerized axial tomography play the most important elements for its diagnosis, while the TNM and Bismuth-Corlette classification are essential for the correct planning of treatment.

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