Klatskin Tumor Explained in Plain Language

What a Klatskin tumor is

It is a malignant tumor of the bile ducts located at the liver hilum, where the right and left hepatic ducts join into the common duct. It is also called perihilar or hilar cholangiocarcinoma. The name comes from Dr. Gerald Klatskin, who described these tumors in 1965^[1].

The liver hilum is a very tight space: branches of the portal vein and hepatic artery, which supply the liver, run right next to the ducts. That is why even a small tumor here quickly blocks bile flow and can involve the blood vessels.

For the work-up and possible operations, see the page on Klatskin tumor.

What the Bismuth–Corlette types mean

Reports often state the tumor type according to the Bismuth–Corlette classification. It shows how far the tumor extends along the ducts^[2][3]:

• type I — the tumor is in the common hepatic duct, below the junction of the right and left ducts;
• type II — it reaches the junction;
• types IIIa and IIIb — it extends into the right (IIIa) or left (IIIb) hepatic duct;
• type IV — it extends into the ducts of both halves of the liver or has several foci.

The type determines which part of the liver has to be removed together with the ducts. But the type alone does not answer whether surgery is possible: involvement of the blood vessels and how much healthy liver will remain also matter.

How it shows itself

Most often as painless jaundice. The skin and the whites of the eyes turn yellow, the urine darkens, the stool becomes pale, and itching appears. Weakness and weight loss often follow. If infection develops in the ducts where bile stagnates, which is cholangitis, a high fever with chills and pain in the upper right abdomen appear.

Why this tumor is difficult to operate on

To remove the tumor completely, it is usually necessary to remove not only the ducts but also part of the liver, often a large part. The main goal is clear margins, meaning no tumor cells at the edges of the removed specimen. Even in the classic small surgical series by Bismuth and colleagues, patients with clear margins lived longer and had fewer recurrences than those whose tumor could not be removed completely^[3].

Radical surgery is the only treatment that offers a chance of cure^[4]. But after part of the liver is removed, enough healthy liver with its own blood supply and duct must remain, and in people with jaundice the liver works less well. That is why patients are often prepared for this operation.

How patients are prepared for surgery

Preparation can take several weeks and involves several steps.

• Bile drainage. It is not used for everyone: drainage increases the risk of infectious complications after surgery, so it is not recommended routinely^[6]. Bile drainage is restored if there is cholangitis, if the part of the liver that will remain is small, or if the jaundice is severe. It is the part of the liver that will remain that gets drained^[4][6]. This is done endoscopically or through the skin. A small randomized trial comparing these two methods was stopped early: overall mortality was higher in the group drained through the skin, mostly after surgery, although complications of the drainage itself were equally common in both groups. The authors urged caution in interpreting this result^[5]. That is why whether drainage is needed, and which method to use, is decided by the team together with the surgeon planning the operation.
• Enlarging the future liver remnant. If the part that will remain is too small, the branch of the portal vein to the part that will be removed is blocked before surgery^[4]. The part that will remain then grows over several weeks.
• Imaging before drainage. CT and MRI of the ducts are best done before a drain or stent is placed, because afterwards the tumor margins are harder to assess^[4].

These procedures are performed by endoscopists and interventional radiologists. We organize this stage and decide which duct to drain. I perform liver surgery together with a team of surgical oncologists.

If surgery is not possible

The most important thing is to restore bile drainage reliably. This is done with an endoscopic stent or a drain through the skin. It reduces jaundice and itching and improves quality of life^[6]. A metal stent is usually placed only once inoperability has been confirmed, because while surgery is still an option it may get in the way^[6].

For patients in reasonably good condition, the standard first-line treatment is chemotherapy combined with immunotherapy. Molecular testing of the tumor tissue is advised before or during first-line treatment: if the disease progresses, its results may make targeted drugs an option^[4]. So if the tumor cannot be removed, it is worth asking for this test.

For selected patients with an inoperable tumor that has not spread, a few specialized centers worldwide perform liver transplantation after chemoradiotherapy under a strict protocol. It is not considered a standard treatment^[4].

What to do if a Klatskin tumor is suspected

1. Have CT and MRI before drainage if possibleAnd show them to a surgeon before a stent is placed: this makes it easier to choose the right approach.
2. Know the warning signsJaundice with a fever or chills: go to a hospital within 1–2 hours. Worsening jaundice or a drain that has stopped working: see a doctor the same day. Confusion, dizziness, low blood pressure: call 103.
3. Ask for an assessment at an experienced centerSurgery for this tumor is complex, and decisions about drainage and the extent of resection are best made together with the surgeon who will operate.
4. Send your scans and discharge summariesThe online consultation is free. We discuss a treatment plan after a full work-up and a multidisciplinary team review.

Sources

1. Klatskin G. Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis. An unusual tumor with distinctive clinical and pathological features. Am J Med. 1965;38:241–256. PMID 14256720
2. Bismuth H, Corlette MB. Intrahepatic cholangioenteric anastomosis in carcinoma of the hilus of the liver. Surg Gynecol Obstet. 1975;140(2):170–178. PMID 1079096
3. Bismuth H, Nakache R, Diamond T. Management strategies in resection for hilar cholangiocarcinoma. Ann Surg. 1992;215(1):31–38. PMID 1309988
4. Vogel A, Bridgewater J, Edeline J, et al. Biliary tract cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2023;34(2):127–140. PMID 36372281
5. Coelen RJS, Roos E, Wiggers JK, et al. Endoscopic versus percutaneous biliary drainage in patients with resectable perihilar cholangiocarcinoma: a multicentre, randomised controlled trial. Lancet Gastroenterol Hepatol. 2018;3(10):681–690. PMID 30122355
6. Dumonceau JM, Tringali A, Papanikolaou IS, et al. Endoscopic biliary stenting: indications, choice of stents, and results: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline — Updated October 2017. Endoscopy. 2018;50(9):910–930. PMID 30086596

This article is for general information and does not replace a consultation: whether surgery is possible is decided from your scans and the multidisciplinary team’s conclusion.