Cholangiocarcinoma

Cholangiocarcinoma (bile duct carcinoma) is diagnosed more often. In part, this can be explained by the introduction of modern diagnostic methods, including new imaging techniques and cholangiography. They allow to establish more precisely the localization and prevalence of the tumor process.

Cholangiocarcinoma and other bile duct tumors, which are very rare, are usually malignant. Cholangiocarcinoma mainly affects extrahepatic bile ducts: 60-80% in the portal gates (Klatskin's tumor) and 10-30% in the distal ducts. Risk factors include advanced age, primary sclerosing cholangitis, hepatic duodenal invasion, and choledochal cysts.

Carcinoma can develop at any level of the biliary tree, from small intrahepatic ducts to the common bile duct. Clinical manifestations and methods of treatment depend on the place of development of the tumor. Surgical treatment is ineffective, mainly due to the low availability of the tumor; nevertheless, there is more and more data on the need to assess the resectability of the tumor in all patients, since the operation, although it does not provide a cure, improves the quality of life of patients. With an inoperable tumor, the argument in favor of X-ray and endoscopic interventions is the elimination of jaundice and itching in dying patients.

Cholangiocarcinoma is associated with ulcerative colitis in combination with or without sclerosing cholangitis. In most cases, cholangiocarcinoma develops against the background of primary sclerosing cholangitis (PSC), accompanied by ulcerative colitis. In patients with primary sclerosing cholangitis combined with ulcerative colitis and colorectal cancer, the risk of developing cholangiocarcinoma is higher than in patients without intestinal tumors.

With congenital cystic diseases, the risk of developing cholangiocarcinoma is increased in all members of the patient's family. Congenital cystic diseases include congenital fibrosis of the liver, cystic enlargement in the intralhepatic ducts (Caroli syndrome), choledocha cyst, polycystic liver and microgamaroma (von Meyenberg complexes). The risk of developing cholangiocarcinoma is also increased with biliary cirrhosis due to atresia of the bile duct.

Invasion of the liver with trematodes in people of Eastern origin can be complicated by intra-hepatic (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 considerable parasitic infestation of the bile ducts near the liver gates.

The invasion of Opistorchis viverrini is of the greatest importance in Thailand, Laos and the western part of Malaysia. These parasites produce carcinogens and free radicals that cause DNA changes and mutations, and also stimulate the proliferation of the epithelium of the intrahepatic bile ducts.

The risk of developing carcinomas of extrahepatic bile ducts after 10 years or more after cholecystectomy is significantly reduced, which indicates a possible association of the tumor with gallstones.

The development of malignant tumors of the biliary tract has no direct connection with cirrhosis, with the exception of biliary cirrhosis.

[1], [2], [3], [4], [5], [6], [7]

Symptoms of cholangiocarcinoma

Patients with cholangiocarcinoma usually complain of skin itching and painless obstructive jaundice (typical for patients aged 50-70 years). Liver tumors can cause only vague abdominal pain, anorexia and weight loss. Other signs may include aholy stools, palpable formation, hepatomegaly, or a strained gallbladder (a symptom of Courvoisier in distal lesions). Pain can resemble a biliary colic (reflex biliary obstruction) or be permanent and progressive. The development of sepsis is uncharacteristic, but it can be provoked by ERCP.

In patients with gall bladder carcinoma, the symptoms of the disease vary from random tumor diagnosis during cholecystectomy performed due to pain and cholelithiasis (70-90% have stones) to a common disease with persistent pain, weight loss and volumetric abdominal cavity formation.

Symptoms of cholangiocarcinoma

Diagnosis of cholangiocarcinoma

Suspicion of cholangiocarcinoma occurs with an unexplained etiology of biliary obstruction. Laboratory studies reflect the degree of cholestasis. Diagnosis is based on ultrasound or CT. If these methods do not allow verifying the diagnosis, it is necessary to perform magnetic resonance cholangiopancreatography (MRCP) or ERCP with percutaneous transhepatic cholangiography. In some cases, ERCP does not only diagnose the tumor, but also allows the tissue to be biopsy bristled, which provides histological diagnosis without fine needle biopsy under the supervision of ultrasound or CT. CT with contrasting also helps in diagnosis.

Gallbladder carcinomas are better diagnosed with CT than with ultrasound. An open laparotomy is necessary to establish the stage of the disease that determines the amount of treatment.

Diagnosis of cholangiocarcinoma

[8], [9], [10]

Treatment of cholangiocarcinoma

Stenting or surgical shunting of obstruction reduces skin itching, jaundice and in some cases fatigue.

Cholangiocarcinoma of the lobule of the liver, confirmed at CT, requires percutaneous or endoscopic (with ERCP) stenting. The distally located cholangiocarcinoma is an indication for endoscopic stenting. If cholangiocarcinoma is limited, an evaluation of resectability with resection of the liver portal ducts or pancreatoduodenal resection is performed during the operation. Adjuvant chemotherapy and radiotherapy with cholangiocarcinoma give encouraging results.

In many cases, gallbladder carcinomas are subject to symptomatic therapy.

Treatment of cholangiocarcinoma