National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Klatskin tumor


Other Names:
Klatskin's tumor; Perihilar cholangiocarcinoma
Categories:
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.[1][2][3][4] 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).[3][4] 

The cause of Klatskin tumors is unknown.[2] Studies suggest that a combination of genetic, environmental, and lifestyle factors (multifactorial) likely influence whether a person will develop cholangiocarcinoma. Because Klatskin tumors are often discovered after they have spread, they can be challenging to treat.[3] Surgical removal of the tumor and relief of bile duct blockage are the main goals of treatment.[1][2][4]
Last updated: 10/13/2016
The symptoms associated with Klatskin tumors are usually due to blocked bile ducts. Symptoms may include:[1][2][3]
  • Jaundice
  • Itching
  • Light colored stools and/or dark urine
  • Abdominal pain
  • Loss of appetite / weight loss
  • Fever
  • Nausea / vomiting
Last updated: 10/13/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cholangiocarcinoma
Bile duct cancer
0030153
Extrahepatic cholestasis 0012334
Jaundice
Yellow skin
Yellowing of the skin
[ more ] 		0000952
30%-79% of people have these symptoms
Hepatomegaly
Enlarged liver
0002240
Lymphadenopathy
Swollen lymph nodes
0002716
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Fatigue
Tired
Tiredness
[ more ] 		0012378
Fever 0001945
Venous thrombosis
Blood clot in vein
0004936
Weight loss 0001824
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Last updated: 7/1/2020
Most Klatskin tumors are diagnosed at an advanced stage.[1][2][3][4] The best long-term results are achieved with surgical intervention.[4] The median survival of patients with non-resectable Klatskin tumors after palliative drainage is two to eight months.[2] Complications include recurring bacterial cholangitis and/or liver failure (cirrhosis).[2] The aim of palliative treatment is improvement in the patient’s quality of life. This includes treating cholestasis and cholangitis, which secondarily prolongs survival.[5][6]
Last updated: 10/13/2016

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Autoimmune cholangitis and primary biliary non-Hodgkin's lymphoma are differential diagnoses of Klatskin tumors.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Klatskin tumor. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • Genetics Home Reference (GHR) contains information on Klatskin tumor. This website is maintained by the National Library of Medicine.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Klatskin tumor. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • My mom has been diagnosed with a Klatskin tumor. They tried to remove it but were unable to do so. What symptoms might develop as her condition progresses? Her eyesight has become blurred. Could this be related to her condition? See answer


  1. Bile Duct (Cholangiocarcinoma) Cancer. American Cancer Society. November 1, 2014; http://www.cancer.org/cancer/bileductcancer/detailedguide/.
  2. Darwin PE. Cholangiocarcinoma. Medscape Reference. August 9, 2016; http://emedicine.medscape.com/article/277393-overview.
  3. Cholangiocarcinoma. Genetics Home Reference (GHR). August 2016; https://ghr.nlm.nih.gov/condition/cholangiocarcinoma.
  4. Furuse J. Klatskin tumor. Orphanet. September 2012; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=99978.
  5. Lillemoe KD. Klatskin tumors. Surgical Treatment: Evidence-Based and Problem-Oriented. 2001;
  6. Witzigmann H, Wiedmann M, Wittekind C, Mossner J, Hauss J. Therapeutical Concepts and Results for Klatskin Tumors. Dtsch Arztebl Int. Feb 2008; 105(9):156-161. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2696740/?tool=pubmed.