Cholangiocarcinoma is a malignant tumor that is found in the bile ducts. It has three main localizations: intrahepatic, extrahepatic (i.e., perihilar, or Klatskin tumors), or extrahepatic. The most common type is extrahepatic, and the least common type is the intrahepatic tumor.
Cholangiocarcinoma arises from epithelial cells lining the biliary tract.
Biliary tract obstruction can occur due to mass effect of cholangiocarcinoma. If not treated, it can lead to life-threatening cholangitis. Signs that indicate obstruction include fatigue, jaundice, pale stools, itching, and dark urine. Infection can be recognized with Charcot's Triad.
The etiology of cholangiocarcinoma is not well understood, but it is thought to be caused by long-standing inflammation, such as that seen in primary sclerosing cholangitis and liver fluke infection. This inflammation induces cellular proliferation and hyperplasia, which leads to malignant transformation.
Liver fluke infections, such as clonorchiasis and opisthorchiasis, have been associated with cholangiocarcinoma. Other infections that can also be linked with cholangiocarcinoma include HIV and H. pylori.
One-third of patients with cholangiocarcinoma can present with weight loss.
Abdominal pain is commonly seen in the later stages of the disease and is usually characterized by a dull ache on the right upper quadrant.
Fatigue is the most common symptom experienced in the early stages of cholangiocarcinoma.
The most common symptom experienced by patients with late cholangiocarcinoma is jaundice. It occurs due to cholestasis secondary to obstruction.
On histology, cholangiocarcinoma characterized by infiltrating neoplastic glands.
On histology, cholangiocarcinoma is characterized by a desmoplastic stroma. This finding is characterized by an expanded matrix production with the remodeling of connective tissue structures within a tumor, not unlike chronic-active scarring.
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