[Intra-hepatic cholangiocarcinoma]

Intra-hepatic cholangiocarcinoma (IHCC) is a rare tumor which arises from the epithelial cells of the intra-hepatic bile ducts; it may develop in a healthy liver and bile ducts or in bile ducts with malignant predisposition (Caroli's syndrome, primary sclerosing cholangitis). It has the worst prognosis of any tumor arising in the liver. Unlike hepatocellular carcinoma, no predisposing factors or high-risk populations have been demonstrated for cholangiocarcinoma other than intraphepatic choledocholithiasis such as is seen in east Asian populations. The most common clinical sign is a palpable tumor mass emphasizing that the tumor is usually detected at an advanced stage. CT scanning yields much clinical information but ultrasound-guided needle biopsy is necessary for diagnosis. Aggressive surgical resection is the only treatment modality which has afforded even slight prolongation of survival; hepatic resection must be large with uninvolved resection margins. When an IHCC is deemed resectable (localized tumor without hepatic metastases or intrahepatic or extrahepatic lymph node spread), pre-operative tumor embolization may be useful; when jaundice is present, percutaneous drainage of the dilated biliary system of the liver to be spared may also be necessary. Neither adjuvant nor neo-adjuvant chemotherapy or radiotherapy have shown proof of efficacity. Cholangiocarcinoma complicates sclerosing cholangitis in 10-15% of cases and is very difficult to diagnose. IHCC may also develop in Caroli's syndrome, where it is commonly found incidentally on pathologic examination of a resection specimen after surgery for a complication of the disease.