Klatskin tumor

Other Names: Klatskin's tumor; Perihilar cholangiocarcinoma Klatskin tumors are are a type of cholangiocarcinoma that begins in an area called the hilum, where the left and right bile ducts join and leave the liver. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases.

Epidemiology

Cholangiocarcinoma affects 8,000 people each year in the United States. This type of cancer occurs much more frequently in Southeast Asian countries such as Thailand, where it is related to infection with a parasite that is common there. For unknown reasons, cholangiocarcinoma occurs slightly more often in men than in women.

Cause

The cause of Klatskin tumors is unResearchers have also investigated inherited variations in several genes as possible risk factors for cholangiocarcinoma. These genetic changes, which are classified as germline mutations, are present in essentially all of the body's cells. However, no specific inherited changes have been found to be major risk factors for this disease.

Several non-genetic risk factors for cholangiocarcinoma have been identified. These include a bile duct disease called primary sclerosing cholangitis, bile duct stones or cysts, and exposure to certain chemical toxins used in manufacturing. In Southeast Asia, infection with parasitic worms that live in the human bile ducts greatly increase the risk of developing cholangiocarcinoma.

Other risk factors that have been studied include long-term infection with viral hepatitis B or C, scarring of the liver (cirrhosis), and chronic diseases such as inflammatory bowel disease and diabetes. Researchers suspect that certain lifestyle factors, including smoking, alcohol use, and obesity, may also contribute to the risk of developing cholangiocarcinoma.

Studies suggest that a combination of genetic, environmental, and lifestyle factors influence whether a person will develop cholangiocarcinoma. However, most people who develop the disease do not have any of the identified risk factors.Studies suggest that a combination of genetic, environmental, and lifestyle factors (multifactorial) likely influence whether a person will develop cholangiocarcinoma.

Inheritance

Cholangiocarcinoma is not inherited. Studies suggest that blood relatives of a person with cholangiocarcinoma may have an increased risk of developing this cancer compared with the general population. However, most people with cholangiocarcinoma do not have a family history of the disease.

Signs and symptoms

Symptoms usually don't present until advanced stages of disease, when jaundice is the most common feature. Other symptoms include abdominal pain, unintentional weight loss, and a general feeling of being unwell (malaise). The symptoms associated with Klatskin tumors are usually due to blocked bile ducts. Symptoms may include:

• Jaundice
• Itching
• Light colored stools and/or dark urine
• Abdominal pain
• Loss of appetite / weight loss
• Fever
• Nausea / vomiting

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Cholangiocarcinoma(Bile duct cancer)
• Extrahepatic cholestasis
• Jaundice(Yellow skin)

30%-79% of people have these symptoms

• Hepatomegaly(Enlarged liver)
• Lymphadenopathy(Swollen lymph nodes)

5%-29% of people have these symptoms

• Abdominal pain(Pain in stomach)
• Fatigue(Tired)
• Fever
• Venous thrombosis(Blood clot in vein)
• Weight loss

Diagnosis

Levels of the tumor markers carbohydrate antigen 19-9 (CA 19-9), carcinoembryonic antigen (CEA) and CA 125 are abnormally high in the bloodstreams of patients with intrahepatic cholangiocarcinoma and Klatskin tumor. The serum CA 19-9 in particular may be very high.

The ultrasonography (and the use of Doppler modes) permit definitive diagnosis of a large number of lesions and the involvement of hepatic hilum, but it is less sensitive than CT or MRI in detecting focal lesions. Ultrasonography always detects dilatation of the bile ducts, but more rarely the tumor itself.

Magnetic resonance cholangiopancreatography (MRCP) is a good non-invasive alternative to these other procedure. This technique demonstrates hepatic parenchyma and it's accurate for detecting nodular carcinomas and infiltrating lesions.

Treatment

Because Klatskin tumors are often discovered after they have spread, they can be challenging to treat. Surgical removal of the tumor and relief of bile duct blockage are the main goals of treatment.

Prognosis

Most Klatskin tumors are diagnosed at an advanced stage. The best long-term results are achieved with surgical intervention. The median survival of patients with non-resectable Klatskin tumors after palliative drainage is two to eight months.

Complications include recurring bacterial cholangitis and/or liver failure (cirrhosis).The aim of palliative treatment is improvement in the patient’s quality of life. This includes treating cholestasis and cholangitis, which secondarily prolongs survival.

NIH genetic and rare disease info

• NIH info on Klatskin tumor

Klatskin tumor is a rare disease.

Klatskin tumor Resources
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