Klatskin's tumor

Klatskin's Tumor Klatskin's tumor, also known as a perihilar cholangiocarcinoma, is a rare type of cancer that occurs at the confluence of the right and left hepatic bile ducts. This tumor is named after Dr. Gerald Klatskin, who first described it in 1965. Klatskin's tumor is a subtype of cholangiocarcinoma, which is a cancer of the bile duct system.

Epidemiology

Klatskin's tumors are rare, with an incidence of approximately 1-2 cases per 100,000 people annually. They are more common in individuals over the age of 60 and have a slight male predominance. Risk factors include primary sclerosing cholangitis, liver fluke infection, and choledochal cysts.

Pathophysiology

Klatskin's tumors arise at the hepatic hilum, where the right and left hepatic ducts join to form the common hepatic duct. The tumor can cause obstruction of bile flow, leading to jaundice and liver dysfunction. The exact cause of Klatskin's tumor is not well understood, but it is believed to involve genetic mutations and chronic inflammation of the bile ducts.

Clinical Presentation

Patients with Klatskin's tumor typically present with painless jaundice, dark urine, pale stools, and pruritus. Other symptoms may include weight loss, abdominal pain, and fatigue. Due to the location of the tumor, symptoms often arise from bile duct obstruction.

Diagnosis

Diagnosis of Klatskin's tumor involves a combination of imaging studies and biopsy. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) are commonly used to visualize the bile ducts. A biopsy may be obtained during ERCP or through percutaneous approaches.

Staging

Staging of Klatskin's tumor is based on the Bismuth-Corlette classification, which categorizes tumors based on their anatomical location and extent of ductal involvement. Staging is crucial for determining the appropriate treatment strategy.

Treatment

The primary treatment for Klatskin's tumor is surgical resection, which may involve a hepatectomy and bile duct reconstruction. However, many patients are not candidates for surgery due to the advanced stage of the disease at diagnosis. In such cases, palliative treatments such as biliary stenting and chemotherapy may be used.

Prognosis

The prognosis for Klatskin's tumor is generally poor, with a 5-year survival rate of less than 20%. Early detection and surgical resection offer the best chance for long-term survival.

Research and Future Directions

Research into Klatskin's tumor is ongoing, with studies focusing on the molecular mechanisms of tumor development and potential targeted therapies. Advances in imaging and surgical techniques also hold promise for improving outcomes.

See Also

• Cholangiocarcinoma
• Bile duct cancer
• Hepatectomy

External Links

• [American Cancer Society: Bile Duct Cancer Overview](https://www.cancer.org/cancer/bile-duct-cancer.html)
• [National Cancer Institute: Bile Duct Cancer Treatment](https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq)

NIH genetic and rare disease info

• NIH info on Klatskin's tumor

Klatskin's tumor is a rare disease.