Klatskin tumor (perihilar cholangiocarcinoma)

What this tumor is and why it develops

Among cholangiocarcinomas, the perihilar tumor is the most common. It is named after the physician who described it in 1965. Depending on how far the tumor extends into the right and left hepatic ducts, it is divided into types by the Bismuth–Corlette classification, from involvement of the common hepatic duct only to extension into the ducts of both halves of the liver. The type determines which part of the liver will have to be removed.

In most patients the cause is unknown. Known risk factors are primary sclerosing cholangitis, bile duct cysts, stones in the ducts and liver flukes (opisthorchiasis, clonorchiasis). Tumors of the intrahepatic ducts themselves are described separately, on the page intrahepatic cholangiocarcinoma.

Are there symptoms

The main sign is painless jaundice: yellowing of the skin and the whites of the eyes, dark urine, pale stools, itchy skin. Weakness, loss of appetite and weight loss often follow. If infection develops in the ducts where bile stagnates (cholangitis), fever with chills and pain under the right ribs appear; this condition requires urgent treatment.

Jaundice has many causes, including bile duct stones and benign strictures. That is why the diagnosis is made by examinations, not by symptoms. More about jaundice itself is on the page malignant obstructive jaundice.

Examinations needed

• Contrast-enhanced CT and MRI with MR cholangiography, ideally before a drain or stent is placed: a drain distorts the picture and makes the extent of the tumor along the ducts harder to assess.
• Vascular assessment: whether branches of the portal vein and hepatic artery are involved, which determines which half of the liver can be preserved.
• Cytology or biopsy during endoscopic examination of the bile ducts (ERCP, cholangioscopy). A negative result does not rule out cancer, so surgery is sometimes performed on the basis of the overall findings. Percutaneous biopsy of the tumor is usually not performed, especially if transplantation is being considered, because of the risk of spreading tumor cells.
• Ruling out IgG4-related cholangitis, a benign inflammation that mimics a tumor but is treated with medication.
• Tumor marker CA 19-9, assessed after the jaundice has subsided, because bile stasis itself raises it.
• Volumetry: calculation of the liver volume that will remain after surgery.
• Chest CT and, when indicated, PET-CT, to rule out distant metastases.

When surgery is possible

Surgery is considered when the tumor can be removed with clear duct margins and enough liver will remain, with its own vascular pedicle and a duct to which the bowel can be joined. Vascular involvement is not always an obstacle: sometimes a segment of the portal vein is removed together with the tumor and blood flow is reconstructed. Distant metastases and involvement of distant lymph nodes usually make radical surgery inappropriate.

Before a major resection in the presence of marked jaundice, cholangitis or a small future liver remnant, bile drainage is usually restored first. The drain is placed in the part of the liver that will remain, endoscopically or through the skin. These procedures are performed by endoscopists and interventional radiologists; we organize this stage and determine which duct should be drained. If the future liver remnant is small, portal vein embolization is also performed to enlarge it.

If surgery is not possible now

The most important thing is to restore bile drainage reliably: for this a stent is placed endoscopically or a drain through the skin. A metal stent is placed only once unresectability has been confirmed by a surgeon; while surgery or transplantation is still an open question, a plastic stent or a drain is used. This reduces jaundice, itching and the risk of cholangitis and allows systemic therapy to begin. The first-line standard for patients in good general condition is chemotherapy combined with immunotherapy; depending on the molecular analysis of the tumor, targeted drugs may be available.

For selected patients with an unresectable tumor without spread, a few specialized centers worldwide perform liver transplantation after chemoradiotherapy under a strict protocol. Whether this route is suitable is determined by the transplant center. The final plan is determined by the MDT.

How the operation is performed

The standard extent is removal of the extrahepatic bile ducts together with the right or left half of the liver (sometimes extended), the caudate lobe and the lymph nodes of the hepatoduodenal ligament. The caudate lobe is removed because its small ducts drain directly into the tumor area. Removing only a segment of the duct without the liver usually does not achieve clear margins.

During surgery the duct margins are examined urgently under the microscope; if tumor is found in them, the duct is resected further where possible. Bile drainage is restored by joining the ducts of the remaining liver to a loop of small bowel.

Because of the complexity of the reconstruction, the operation is mostly performed open. Laparoscopically only in selected cases, when the tumor’s location and the extent of resection allow.

Recovery after surgery

Care follows the enhanced recovery after surgery (ERAS) protocol: early mobilization, early feeding, controlled pain relief. After this operation the hospital stay is usually longer than after an ordinary liver resection: in the first days liver function, possible bile leakage and signs of infection are monitored. Drains are removed once they are no longer needed.

Adjuvant chemotherapy is usually given after surgery. Its regimen is determined by the medical oncologist based on the pathology report.

Follow-up

For the first years, CT or MRI and the tumor marker CA 19-9 are done regularly, every few months, then less often; the schedule is set by the doctor. The duct-to-bowel anastomosis is also monitored: a narrowing of this connection or cholangitis shows up as fever, jaundice and pain, and requires seeing a doctor without waiting for the scheduled visit.

What to bring to the consultation

• CT and MRI with MR cholangiography discs and reports, especially those done before a drain or stent was placed;
• reports of ERCP, cholangioscopy, percutaneous drainage;
• cytology or histology reports, slides;
• blood biochemistry with bilirubin, tumor marker CA 19-9;
• discharge summaries from other hospitals, if you have already been treated.

If some materials are missing, that is no reason to postpone the consultation: some examinations can be done on site.

The consultation is free of charge. How it goes and how long it takes is described on the page “Oncologist Consultation”.