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Cholangiocarcinoma
Last reviewed: 04.07.2025
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Cholangiocarcinoma (bile duct carcinoma) is being diagnosed more and more often. This can be partly explained by the introduction of modern diagnostic methods, including new imaging techniques and cholangiography. They allow more accurate localization and spread of the tumor process.
Cholangiocarcinoma and other bile duct tumors, although rare, are usually malignant. Cholangiocarcinoma predominantly affects the extrahepatic bile ducts: 60-80% at the porta hepatis (Klatskin tumor) and 10-30% in the distal duct. Risk factors include older age, primary sclerosing cholangitis, liver fluke invasion, and choledochal cysts.
Carcinoma may develop at any level of the biliary tree, from small intrahepatic ducts to the common bile duct. Clinical manifestations and treatment methods depend on the site of tumor development. Surgical treatment is ineffective, mainly due to the inaccessibility of the tumor; however, there is increasing evidence for the need to assess the resectability of the tumor in all patients, since surgery, although not curative, improves the quality of life of patients. In the case of inoperable tumors, the argument in favor of X-ray surgical and endoscopic interventions is the elimination of jaundice and pruritus in dying patients.
Cholangiocarcinoma is associated with ulcerative colitis, with or without sclerosing cholangitis. In most cases, cholangiocarcinoma develops in the setting of primary sclerosing cholangitis (PSC) accompanied by ulcerative colitis. Patients with primary sclerosing cholangitis combined with ulcerative colitis and colorectal tumor have a higher risk of developing cholangiocarcinoma than patients without a bowel tumor.
In congenital cystic diseases, the risk of developing cholangiocarcinoma is increased in all family members of the patient. Congenital cystic diseases include congenital liver fibrosis, cystic dilatations in the intrahepatic ducts (Caroli syndrome), common bile duct cyst, polycystic liver disease, and microhamartoma (von Meyenberg complexes). The risk of developing cholangiocarcinoma is also increased in biliary cirrhosis due to biliary atresia.
Liver invasion by trematodes in people of oriental origin may be complicated by intrahepatic (cholangiocellular) cholangiocarcinoma. In the Far East (China, Hong Kong, Korea, Japan), where Clonorchis sinensis is most common, cholangiocarcinoma accounts for 20% of all primary liver tumors. These tumors develop with significant parasite invasion of the bile ducts near the porta hepatis.
Opistorchis viverrini infestations are most important in Thailand, Laos and western Malaysia. These parasites produce carcinogens and free radicals that cause DNA changes and mutations, and stimulate proliferation of the intrahepatic bile duct epithelium.
The risk of developing extrahepatic bile duct carcinoma 10 years or more after cholecystectomy is significantly reduced, indicating a possible association between the tumor and gallstones.
The development of malignant tumors of the bile ducts has no direct connection with cirrhosis, with the exception of biliary cirrhosis.
[ Symptoms of Cholangiocarcinoma
Patients with cholangiocarcinoma typically present with pruritus and painless obstructive jaundice (usually in patients aged 50–70 years). Porta hepatis tumors may cause only vague abdominal pain, anorexia, and weight loss. Other signs may include acholic stools, a palpable mass, hepatomegaly, or a tense gallbladder (Courvoisier's sign in distal lesions). The pain may resemble biliary colic (reflex biliary obstruction) or be persistent and progressive. Sepsis is uncommon but may be triggered by ERCP.
In patients with gallbladder carcinoma, symptoms range from incidental diagnosis of the tumor during cholecystectomy performed because of pain and cholelithiasis (70-90% have stones) to widespread disease with persistent pain, weight loss, and abdominal mass.
What's bothering you?
Diagnosis of cholangiocarcinoma
Cholangiocarcinoma is suspected when the etiology of biliary obstruction is unexplained. Laboratory tests reflect the degree of cholestasis. Diagnosis is based on ultrasound or CT. If these methods do not allow to verify the diagnosis, magnetic resonance cholangiopancreatography (MRCP) or ERCP with percutaneous transhepatic cholangiography should be performed. In some cases, ERCP not only diagnoses the tumor, but also allows for a tissue brush biopsy, which provides histological diagnosis without fine-needle biopsy under ultrasound or CT control. CT with contrast also helps in diagnosis.
Gallbladder carcinomas are better detected by CT than by ultrasound. Open laparotomy is necessary to establish the stage of the disease, which determines the extent of treatment.
Diagnosis of cholangiocarcinoma
What do need to examine?
Treatment of cholangiocarcinoma
Stenting or surgical bypass of the obstruction reduces itching, jaundice, and in some cases fatigue.
Cholangiocarcinoma of the hepatic porta, confirmed by CT, requires percutaneous or endoscopic (with ERCP) stenting. Distal cholangiocarcinoma is an indication for endoscopic stenting. If cholangiocarcinoma is limited, resectability is assessed during surgery with resection of the hepatic porta ducts or pancreaticoduodenectomy. Adjuvant chemotherapy and radiotherapy for cholangiocarcinoma give encouraging results.
In many cases, gallbladder carcinomas are treated symptomatically.