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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
Age of onset
Adult
ICD-10
C24.0
Inheritance
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.
X-linked
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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
Categories
Digestive Diseases; Rare Cancers
Summary
Klatskin
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]
Symptoms
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:
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 |
Related diseases
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.
|
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.
References
- Bile Duct (Cholangiocarcinoma) Cancer. American Cancer Society. November 1, 2014; https://www.cancer.org/cancer/bileductcancer/detailedguide/.
- Darwin PE. Cholangiocarcinoma. Medscape Reference. August 9, 2016; https://emedicine.medscape.com/article/277393-overview.
- Cholangiocarcinoma. Genetics Home Reference (GHR). August 2016; https://ghr.nlm.nih.gov/condition/cholangiocarcinoma.
- Furuse J. Klatskin tumor. Orphanet. September 2012; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=99978.
- Lillemoe KD. Klatskin tumors. Surgical Treatment: Evidence-Based and Problem-Oriented. 2001;
- 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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