Rare Gastroenterology News

Disease Profile

Klatskin tumor

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

1-9 / 100 000

US Estimated

Europe Estimated

Age of onset






Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Klatskin's tumor; Perihilar cholangiocarcinoma


Digestive Diseases; Rare Cancers


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]


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

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.

Medical Terms Other Names
Learn More:
80%-99% of people have these symptoms
Bile duct cancer
Extrahepatic cholestasis
Yellow skin
Yellowing of the skin

[ more ]

30%-79% of people have these symptoms
Enlarged liver
Swollen lymph nodes
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain

[ more ]


[ more ]

Venous thrombosis
Blood clot in vein
Weight loss

Learn more

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.


  1. Bile Duct (Cholangiocarcinoma) Cancer. American Cancer Society. November 1, 2014; https://www.cancer.org/cancer/bileductcancer/detailedguide/.
  2. Darwin PE. Cholangiocarcinoma. Medscape Reference. August 9, 2016; https://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; https://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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2696740/?tool=pubmed.

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