Cholangiocarcinoma (CC)

Categories: Cancer diseases, Liver diseases, Nephrological diseases, Rare diseases, Skin diseases

Aliases & Classifications for Cholangiocarcinoma

MalaCards integrated aliases for Cholangiocarcinoma:

Name: Cholangiocarcinoma 56 74 25 58 36 29 54 6 37 17 71
Cholangiocarcinoma, Susceptibility to 56 29
Intrahepatic Cholangiocarcinoma 25 71
Extrahepatic Cholangiocarcinoma 25 71
Adult Primary Cholangiocellular Carcinoma 71
Cholangiocarcinoma of Biliary Tract 25
Perihilar Cholangiocarcinoma 25
Cholangiocellular Carcinoma 25
Distal Cholangiocarcinoma 25
Chlc, Susceptibility to 56
Bile Duct Carcinoma 71
Bile Duct Cancer 58
Chlc 56
Cca 58
Cc 25


Orphanet epidemiological data:

Inheritance: Not applicable; Prevalence: 1-9/100000 (Worldwide),1-9/100000 (Europe); Age of onset: Adult;


Orphanet: 58  
Rare hepatic diseases

External Ids:

OMIM 56 615619
KEGG 36 H00046
MESH via Orphanet 44 D001650 D018281
ICD10 via Orphanet 33 C22.1 C24.0 C24.8 more
UMLS via Orphanet 72 C0206698 C0740277
Orphanet 58 ORPHA70567
MedGen 41 C3810156
UMLS 71 C0206698 C0280725 C0345905 more

Summaries for Cholangiocarcinoma

Genetics Home Reference : 25 Cholangiocarcinoma is a group of cancers that begin in the bile ducts. Bile ducts are branched tubes that connect the liver and gallbladder to the small intestine. They carry bile, which is a fluid that helps the body digest fats that are in food. Bile is made in the liver and stored in the gallbladder before being released in the small intestine after a person eats. Cholangiocarcinoma is classified by its location in relation to the liver. Intrahepatic cholangiocarcinoma begins in the small bile ducts within the liver. This is the least common form of the disease, accounting for less than 10 percent of all cases. Perihilar cholangiocarcinoma (also known as a Klatskin tumor) begins in an area called the hilum, where the right and left major bile ducts join and leave the liver. It is the most common form of the disease, accounting for more than half of all cases. The remaining cases are classified as distal cholangiocarcinomas, which begin in bile ducts outside the liver. The perihilar and distal forms of the disease, which both occur outside the liver, are sometimes grouped together and called extrahepatic cholangiocarcinoma. The three types of cholangiocarcinoma do not usually cause any symptoms in their early stages, and this cancer is usually not diagnosed until it has already spread beyond the bile ducts to other tissues. Symptoms often result when bile ducts become blocked by the tumor. The most common symptom is jaundice, in which the skin and whites of the eyes turn yellow. Other symptoms can include extreme tiredness (fatigue), itching, dark-colored urine, loss of appetite, unintentional weight loss, abdominal pain, and light-colored and greasy stools. These symptoms are described as "nonspecific" because they can be features of many different diseases. Most people who develop cholangiocarcinoma are older than 65. Because this cancer is often not discovered until it has already spread, it can be challenging to treat effectively. Affected individuals can survive for several months to several years after diagnosis, depending on the location of the cancer and how advanced it is.

MalaCards based summary : Cholangiocarcinoma, also known as cholangiocarcinoma, susceptibility to, is related to klatskin's tumor and small cell cancer of the lung, and has symptoms including nausea and vomiting, abdominal pain and constipation. An important gene associated with Cholangiocarcinoma is MIR21 (MicroRNA 21), and among its related pathways/superpathways is MicroRNAs in cancer. The drugs Desflurane and Sevoflurane have been mentioned in the context of this disorder. Affiliated tissues include liver, lymph node and t cells, and related phenotypes are jaundice and biliary tract neoplasm

OMIM : 56 Carcinomas of the biliary tract are aggressive malignancies, with 5-year survival of less than 10%. These carcinomas arise throughout the biliary tree and are anatomically classified as either intrahepatic or extrahepatic cholangiocarcinomas. Gallbladder carcinomas also arise from the biliary tree but have distinct natural histories compared to cholangiocarcinomas, suggesting different underlying tumor biology. Cholangiocarcinoma incidence varies widely between geographic regions, reflecting the impact of different underlying etiologies. In endemic areas, liver fluke infections by O. viverrini and Clonorchis sinensis, both group I carcinogens, represent the major risk factor for cholangiocarcinomas. In nonendemic regions, other risk factors, including choledochal cysts (603003), hepatolithiasis, and primary sclerosing cholangitis (613806), are likely contributors (summary by Chan-on et al., 2013). Overall, the majority of patients lack such identifiable risk factors (summary by Jiao et al., 2013). (615619)

KEGG : 36 Cholangiocarcinoma is a highly malignant neoplasm that carries a poor prognosis and lacks effective therapy. It is the second most common primary hepatic tumor, and it is increasing in incidence and carries a high mortality. The tumor arises from the ductular epithelium of the biliary tree, either within the liver (intrahepatic cholangiocarcinoma: ICC) or more commonly from the extrahepatic bile ducts (extrahepatic cholangiocarcinoma). Several studies have demonstrated mutations resulting in overexpression of K-ras and p53 genes. These genetic alterations are associated with a more aggressive phenotype in this cancer. Many reports have implicated overexpression of the tyrosine kinase proto-oncogenes c-erbB-2 (HER-2/neu) and c-Met, as well as cyclo-oxygenase-2 (COX-2) activity in intrahepatic cholangiocarcinoma.

Wikipedia : 74 Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts.... more...

Related Diseases for Cholangiocarcinoma

Diseases related to Cholangiocarcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1008)
# Related Disease Score Top Affiliating Genes
1 klatskin's tumor 34.4 MIR373 MALAT1
2 small cell cancer of the lung 30.8 MIR21 MALAT1 CCAT2
3 pancreatic ductal adenocarcinoma 30.6 MIR21 MIR148A MIR141 MALAT1 H19 AFAP1-AS1
4 astrocytoma 30.5 SOX2-OT NEAT1 MIR21 H19
5 microvascular complications of diabetes 3 30.3 MIR21 MALAT1
6 kidney cancer 30.2 MIR21 MIR200B MIR141 MALAT1 H19
7 nasopharyngeal carcinoma 30.2 NEAT1 MIR21 MIR141 MALAT1 HULC H19
8 pulmonary hypertension, primary, 1 30.2 MIR204 MALAT1
9 thyroid cancer, nonmedullary, 1 30.1 NEAT1 MALAT1 HULC H19
10 nervous system disease 30.1 MIR373 MIR21 MIR204 MIR200B
11 glioma susceptibility 1 30.0 PCAT1 MIR21 MALAT1 H19
12 tongue squamous cell carcinoma 30.0 MIR21 MALAT1 AFAP1-AS1
13 central nervous system disease 30.0 SOX2-OT MIR21 MIR200B H19
14 pancreatic cancer 29.9 SPRY4-IT1 NEAT1 MIR21 MIR148A MIR141 MALAT1
15 pancreas disease 29.9 MIR373 MIR21 MIR148A MIR141
16 bladder disease 29.9 MIR21 MIR204 MIR148A MIR141
17 laryngeal squamous cell carcinoma 29.9 NEAT1 MIR21 MIR148A MALAT1 H19
18 lung cancer susceptibility 3 29.9 MIR21 MIR200B MIR148A MIR141 MALAT1 H19
19 thyroid gland cancer 29.8 PANDAR MALAT1 H19 AFAP1-AS1
20 squamous cell carcinoma 29.8 SPRY4-IT1 SOX2-OT PCAT1 NEAT1 MALAT1 H19
21 osteogenic sarcoma 29.7 SOX2-OT PCAT1 PANDAR NEAT1 MALAT1 HULC
22 diffuse large b-cell lymphoma 29.7 PANDAR MIR320A MIR21 MIR200B HULC
23 gastrointestinal system disease 29.7 MIR373 MIR21 MIR204 MIR200B MIR148A MIR141
24 myeloma, multiple 29.6 PCAT1 MIR21 MALAT1 H19
25 melanoma 29.5 SPRY4-IT1 SOX2-OT MIR204 MIR200B MIR141 MALAT1
26 gastrointestinal system cancer 29.4 MIR373 MIR21 MIR204 MIR200B MIR148A MIR141
27 renal cell carcinoma, nonpapillary 29.4 SPRY4-IT1 PCAT1 PANDAR MIR21 MIR204 MIR141
28 glioma 29.4 SPRY4-IT1 NEAT1 MIR21 MALAT1 HULC H19
29 hepatocellular carcinoma 29.4 SPRY4-IT1 SOX2-OT PCAT1 PANDAR NEAT1 MIR21
30 bladder cancer 29.2 SPRY4-IT1 PCAT1 PANDAR MIR21 MIR141 MALAT1
31 oral squamous cell carcinoma 29.2 NEAT1 MIR370 MIR21 MIR204 MIR200B MIR148A
32 esophageal cancer 29.1 PCAT1 MIR373 MIR21 MIR204 MIR148A MIR141
33 cervical cancer 29.0 SPRY4-IT1 PCAT1 PANDAR NEAT1 MIR21 MIR204
34 squamous cell carcinoma, head and neck 28.9 SPRY4-IT1 MIR373 MIR21 MIR204 MIR200B MIR148A
35 clear cell renal cell carcinoma 28.9 SPRY4-IT1 PANDAR MIR320A MIR21 MIR204 MALAT1
36 colorectal cancer 28.5 SPRY4-IT1 SOX2-OT PCAT1 PANDAR NEAT1 MIR320A
37 ovarian cancer 28.5 SPRY4-IT1 SOX2-OT NEAT1 MIR21 MIR200B MIR152
38 gastric cancer 28.3 SPRY4-IT1 SOX2-OT PCAT1 PANDAR NEAT1 MIR21
39 lung cancer 28.0 SPRY4-IT1 SOX2-OT PCAT1 PANDAR NEAT1 MIR373
40 breast cancer 27.9 SPRY4-IT1 SOX2-OT PTPN3 PANDAR NEAT1 MIR373
41 prostate cancer 27.5 SPRY4-IT1 PCAT1 NEAT1 MIR370 MIR320A MIR21
42 intrahepatic cholangiocarcinoma 12.9
43 contractural arachnodactyly, congenital 12.5
44 mucinous intrahepatic cholangiocarcinoma 12.4
45 sarcomatous intrahepatic cholangiocarcinoma 12.4
46 combined hepatocellular carcinoma and cholangiocarcinoma 12.4
47 signet ring cell intrahepatic cholangiocarcinoma 12.2
48 bile duct cancer 11.8
49 polyposis, skin pigmentation, alopecia, and fingernail changes 11.8
50 cholangitis, primary sclerosing 11.8

Graphical network of the top 20 diseases related to Cholangiocarcinoma:

Diseases related to Cholangiocarcinoma

Symptoms & Phenotypes for Cholangiocarcinoma

Human phenotypes related to Cholangiocarcinoma:

58 31 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 jaundice 58 31 hallmark (90%) Very frequent (99-80%) HP:0000952
2 biliary tract neoplasm 58 31 hallmark (90%) Very frequent (99-80%) HP:0100574
3 acholic stools 58 31 hallmark (90%) Very frequent (99-80%) HP:0011985
4 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
5 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
6 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
7 abdominal pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0002027
8 anorexia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002039
9 cholangiocarcinoma 31 HP:0030153

Clinical features from OMIM:


UMLS symptoms related to Cholangiocarcinoma:

nausea and vomiting, abdominal pain, constipation, diarrhea, icterus, dyspepsia, heartburn, gastrointestinal gas, malaise

Drugs & Therapeutics for Cholangiocarcinoma

Drugs for Cholangiocarcinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 319)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Desflurane Approved Phase 4 57041-67-5 42113
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
Etomidate Approved Phase 4 33125-97-2 36339 667484
4 Cyclooxygenase 2 Inhibitors Phase 4
5 Cyclooxygenase Inhibitors Phase 4
6 Anti-Inflammatory Agents, Non-Steroidal Phase 4
7 Anesthetics, Inhalation Phase 4
8 Platelet Aggregation Inhibitors Phase 4
9 Analgesics Phase 4
10 Narcotics Phase 4
11 Hypnotics and Sedatives Phase 4
12 Anesthetics, General Phase 4
13 Analgesics, Opioid Phase 4
14 Anesthetics, Intravenous Phase 4
15 Omega 3 Fatty Acid Phase 4
16 Neurotransmitter Agents Phase 4
17 Psychotropic Drugs Phase 4
18 Anti-Anxiety Agents Phase 4
Bismuth Phase 4 7440-69-9 16682734 105143
20 Ulipristal acetate Phase 4 126784-99-4
21 GABA Modulators Phase 4
Arginine Investigational, Nutraceutical Phase 4 74-79-3 6322
Gemcitabine Approved Phase 3 95058-81-4 60750
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2
Atezolizumab Approved, Investigational Phase 3 1380723-44-3
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 3 7487-88-9 24083
Ipilimumab Approved Phase 3 477202-00-9
nivolumab Approved Phase 3 946414-94-4
Cisplatin Approved Phase 3 15663-27-1 2767 441203 84093
Paclitaxel Approved, Vet_approved Phase 2, Phase 3 33069-62-4 36314
Simvastatin Approved Phase 3 79902-63-9 54454
Fluorouracil Approved Phase 3 51-21-8 3385
leucovorin Approved Phase 3 58-05-9 6006 143
Citalopram Approved Phase 3 59729-33-8 2771
Glycine Approved, Nutraceutical, Vet_approved Phase 3 56-40-6 750
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
38 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
39 Hematinics Phase 3
40 Antipsychotic Agents Phase 3
41 Emetics Phase 3
42 Anthelmintics Phase 3
43 Antiparasitic Agents Phase 3
44 Pharmaceutical Solutions Phase 3
45 Calcium, Dietary Phase 2, Phase 3
46 Folfirinox Phase 2, Phase 3
47 Vaccines Phase 3
48 Albumin-Bound Paclitaxel Phase 2, Phase 3
49 Hypolipidemic Agents Phase 3
50 Anticholesteremic Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 630)
# Name Status NCT ID Phase Drugs
1 Prospective, Randomized, Open-label, Controlled Trial of Cyclooxygenase-2 Inhibitor (Celecoxib; Celebrex®) for Adjuvant Anticancer Effect in Patients With Biliary-pancreas Cancer. Unknown status NCT01111591 Phase 4 Cox2 inhibitor (Celecoxib)
2 Study of the Relationship Between the Rate of Residual Platinum in the Blood and the Incidence of Persistent Neurotoxicity in Patients Treated for Gastrointestinal Cancer With Oxaliplatin Unknown status NCT00274885 Phase 4 oxaliplatin
3 Effect of Preoperative Biliary Drainage on Postoperative Complications in Resectable Patients With Hilar Cholangiocarcinoma(Klatskin Tumor) Unknown status NCT00797121 Phase 4
4 Quality of Life in Patients With Unresectable Hilar Cholangiocarcinoma on Palliative Metallic Stent Versus Plastic Stent: A Randomized Control Trial Unknown status NCT00746538 Phase 4
5 Effects of Different Inhalational Anesthetic Agents on the Incidence of Clinical and Subclinical Acute Kidney Injury After Liver Resection Surgery: a Pilot Study Unknown status NCT02174575 Phase 4 Sevoflurane;Desflurane
6 Effects of Preoperative Immunonutrition on Infectious Complication and Th1/Th2 Differentiation in Patients Undergoing Hepatectomy Completed NCT01256047 Phase 4
7 Endoscopic Radiofrequency Ablation for Malignant Biliary Obstruction Completed NCT01758341 Phase 4
8 Efficacy and Cost Analysis of Plastic Stent Compare to Metallic Stent in Unresectable Complex Hilar Cholangiocarcinoma; a Randomized Controlled Trial Completed NCT00721175 Phase 4
9 Covered Versus Uncovered Metal Stents for Management of Distal Malignant Biliary Obstruction? Results of a Randomized Prospective Study. Completed NCT00280709 Phase 4
10 Effects of Preoperative Immunonutrition on Infectious Complication and Th1/Th2 Differentiation in Patients Undergoing Pancreaticoduodenectomy Completed NCT01256034 Phase 4
11 Pilot Study: Randomized Controlled Trial on the Influence of an Oral Nutritional Supplement Rich in Omega-3 Fatty Acids on Functional State and Quality of Life in Malnourished Patients With Gastroenterological Tumors Completed NCT00168987 Phase 4 oral nutritional supplement rich in eicosapentanoic acid
12 Re-intervention for Occluded Biliary Metal Stent in Malignant Distal Bile Duct Obstruction: a Prospective Randomized Multi-center Trial Comparing Covered and Uncovered Metal Stent Completed NCT01315522 Phase 4
13 Etomidate With Meperidine vs Midazolam With Meperidine for Sedation During Endodscopic Retrograde Cholangiopancreatogram (ERCP) Completed NCT02027311 Phase 4 Etomidate;Midazolam;Meperidine
14 Liver Transplant Combined With Neoadjuvant Chemo-radiotherapy in the Treatment of Unresectable Hilar Cholangiocarcinoma. A Prospective Multicenter Study. Recruiting NCT04378023 Phase 4 Neoadjuvant Chemo-radiotherapy
15 Early Oral Versus Enteral Nutrition After Pancreatoduodenectomy for Periampullary Tumors: a Prospective, Randomized, Controlled Clinical Trial Recruiting NCT01642875 Phase 4
16 Clinical Trial of Adjuvant Chemotherapy Followed by Concurrent Chemoradiotherapy Compared With Adjuvant Chemotherapy Alone in Patients With Gallbladder Carcinoma and Extrahepatic Cholangiocarcinoma Unknown status NCT02798510 Phase 3 capecitabine;gemcitabine
17 Oxaliplatin Plus Gemcitabine Versus Capecitabine Alone as Adjuvant Treatment in the Prevention of Recurrence of Intrahepatic Cholangiocarcinoma Unknown status NCT02548195 Phase 3 oxaliplatin and gemcitabine;capecitabine
18 Open-label Observational Study of Plastic Cylindrical Fiber Optic Diffuser (Pioneer Optics) in Photodynamic Therapy for the Management of Cholangiocarcinoma. Unknown status NCT01755013 Phase 2, Phase 3
19 A Randomized Controlled Trial Comparing Endoscopic Biliary Radiofrequency Ablation With Photodynamic Therapy for Inoperable Cholangiocarcinoma Unknown status NCT01739465 Phase 2, Phase 3
20 Unilateral Versus Bilateral Stents for Bismuth Type II and III Malignant Hilar Strictures: A Prospective, Multi-center Randomized Controlled Trial Unknown status NCT00653978 Phase 3
21 A Prospective, Randomized Trial of Early Enteral Feeding After Pylorus Preserving Pancreatoduodenectomy Unknown status NCT00809081 Phase 3
22 A Randomised Clinical Trial Evaluating Adjuvant Chemotherapy With Capecitabine Compared to Expectant Treatment Alone (Observation) Following Surgery for Biliary Tract Cancer Completed NCT00363584 Phase 3 capecitabine
23 Phase III Multicenter Randomized Study Comparing the Effect of Adjuvant Chemotherapy for Six Months With Gemcitabine-Oxaliplatin 85 mg/m2 (GEMOX 85) to Observation in Patients Who Underwent Surgery for Cancer of the Bile Ducts Completed NCT01313377 Phase 3 gemcitabine hydrochloride;oxaliplatin
24 Photodynamic Therapy With S-1 in the Patients With Unresectable Type III or IV Cholangiocarcinoma: A Prospective Randomized Trial With Open Label Control Completed NCT00869635 Phase 3 S-1 Chemotherapy
25 Porfimer Sodium Photodynamic Therapy Plus Stenting Versus Stenting Alone in Patients With Advanced or Metastatic Cholangiocarcinomas and Other Biliary Tract Tumours: a Multicentre, Randomised, Phase Lll Study Completed NCT00513539 Phase 3 porfimer sodium
26 Gemcitabine, Alone or in Combination With Cisplatin, in Patients With Advanced or Metastatic Cholangiocarcinomas and Other Biliary Tract Tumors: A Multicentre, Randomized Phase III Study Completed NCT00262769 Phase 3 cisplatin;gemcitabine hydrochloride
27 Randomized Phase II-III Study of Chemoradiation With Fluorouracil and Cisplatin Versus Chemotherapy (Gemcitabine/Oxaliplatin) in Non Resectable But Non Metastatic Cancer of the Biliary Tract Completed NCT00304135 Phase 2, Phase 3 cisplatin;gemcitabine hydrochloride;oxaliplatin
28 Sequential Preoperative Ipsilateral Hepatic Vein Embolization After Portal Vein Embolization to Induce Further Liver Regeneration in Patients With Hepatobiliary Malignancy Completed NCT00698880 Phase 2, Phase 3
29 A Randomized Phase III Double-Blind Study Of Ondansetron And Dexamethasone Versus Ondansetron And Placebo In The Prophylaxis Of Radiation-Induced Emesis Completed NCT00016380 Phase 3 dexamethasone;ondansetron
30 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
31 A Phase III, Randomised, Multicentre Open-label Study of Active Symptom Control (ASC) Alone or ASC With Oxaliplatin/ 5F-U Chemotherapy for Patients With Locally Advanced/ Metastatic Biliary Tract Cancers Previously Treated With Cisplatin/ Gemcitabine Chemotherapy. Completed NCT01926236 Phase 3 L-folinic acid;5 FU;Oxaliplatin
32 The Efficacy of Levamisole Hcl in Advanced Intrahepatic Cholangiocarcinoma . A Multicenter, Open, Randomized, Prospective Study Recruiting NCT03940378 Phase 3 Levamisole Hydrochloride;Anlotinib Hydrochloride Capsules
33 Bortezomib in Treating Patients With Intrahepatic Cholangiocellular Carcinoma Featuring PTEN Deficiency Recruiting NCT03345303 Phase 3 Bortezomib
34 Hepatic Arterial Infusion Chemotherapy of Irinotecan, Oxaliplatin, 5-Fluorouracil and Leucovorin Versus Systemic Chemotherapy of Gemcitabine and Oxaliplatin for Unresectable Intrahepatic Cholangiocarcinoma Recruiting NCT03771846 Phase 3 irinotecan, oxaliplatin , fluorouracil, and leucovorin;gemcitabine and oxaliplatin
35 Neoadjuvant Chemotherapy With Gemcitabine Plus Cisplatin Followed by Radical Liver Resection Versus Immediate Radical Liver Resection Alone With or Without Adjuvant Chemotherapy in Incidentally Detected Gallbladder Carcinoma After Simple Cholecystectomy or in Front of Radical Resection of BTC (ICC/ECC) - A Phase III Study of the German Registry of Incidental Gallbladder Carcinoma Platform (GR) - The AIO/ CALGP/ ACO- GAIN-Trial - Recruiting NCT03673072 Phase 3 Gemcitabine;Cisplatin;Adjuvant chemotherapy
36 Randomized, Controlled Study to Compare the Efficacy, Safety and Pharmacokinetics of Melphalan/HDS Treatment Given Sequentially Following Cisplatin/Gemcitabine Versus Cisplatin/Gemcitabine in Patients With IntraHepatic Cholangiocarcinoma Recruiting NCT03086993 Phase 2, Phase 3 Cisplatin and Gemcitabine
37 A Randomized, Open, Multi-center Phase IIb/III Clinical Study to Assess the Efficacy and Safety of Surufatinib Compared to Capecitabine in Advanced or Metastatic Biliary Tract Carcinoma (BTC) Patients Recruiting NCT03873532 Phase 2, Phase 3 Surufatinib;Capecitabine
38 A Phase 3 Randomized, Double Blind Study of Pembrolizumab Plus Gemcitabine/Cisplatin Versus Placebo Plus Gemcitabine/Cisplatin as First-Line Therapy in Participants With Advanced and/or Unresectable Biliary Tract Carcinoma Recruiting NCT04003636 Phase 3 Pembrolizumab;Gemcitabine;Cisplatin;Placebo
39 Randomised Phase II/III Study, Assessing the Safety and Efficacy of Modified Folfirinox Versus Gemcis in Locally Advanced, Unresectable and/or Metastatic Bile Duct Tumours Recruiting NCT02591030 Phase 2, Phase 3 GEMCIS;mFolfirinox
40 A Phase III Randomized Trial of Gemcitabine, Cisplatin, and Nab-Paclitaxel Versus Gemcitabine and Cisplatin in Newly Diagnosed, Advanced Biliary Tract Cancers Recruiting NCT03768414 Phase 3 Cisplatin;Gemcitabine Hydrochloride;Nab-paclitaxel
41 A Phase 3 Multicenter, Open-Label, Randomized, Controlled Study of Oral Infigratinib Versus Gemcitabine With Cisplatin in Subjects With Advanced/Metastatic or Inoperable Cholangiocarcinoma With FGFR2 Gene Fusions/Translocations: The PROOF Trial Recruiting NCT03773302 Phase 3 BGJ398;Gemcitabine;Cisplatin
42 A Phase 3, Open-Label, Randomized, Active-Controlled, Multicenter Study to Evaluate the Efficacy and Safety of Pemigatinib Versus Gemcitabine Plus Cisplatin Chemotherapy in First-Line Treatment of Participants With Unresectable or Metastatic Cholangiocarcinoma With FGFR2 Rearrangement (FIGHT-302) Recruiting NCT03656536 Phase 3 Pemigatinib;Gemcitabine;Cisplatin
43 Percutaneous Bilateral Versus Unilateral Metal Stent Placement for Hilar Cholangiocarcinoma : A Prospective Open-label Randomized Controlled Trial Recruiting NCT02108145 Phase 2, Phase 3
44 Phase III Randomized Trial of High Dose Chemoradiation and Systemic Chemotherapy vs Systemic Chemotherapy Alone in Patients With Unresectable Nonmetastatic Cholangiocarcinoma Recruiting NCT02773485 Phase 3 Systemic chemotherapy
45 Adjuvant Chemotherapy With Gemcitabine and Capecitabine Compared to Capecitabine for Biliary Tract Cancer After Curative Resection Recruiting NCT03779035 Phase 3 Gemcitabine;Capecitabine
46 Adjuvant Chemotherapy With Gemcitabine and Cisplatin Compared to Standard of Care After Curative Intent Resection of Cholangiocarcinoma and Muscle Invasive Gall Bladder Carcinoma (ACTICCA-1 Trial) Recruiting NCT02170090 Phase 3 Gemcitabine;Cisplatin;Capecitabine
47 A Phase II/III, Multicenter, Randomized, Placebo-controlled Study of Gemcitabine Plus Cisplatin With or Without Bintrafusp Alfa (M7824) as First-line Treatment of Biliary Tract Cancer Recruiting NCT04066491 Phase 2, Phase 3 Bintrafusp alfa;Placebo;Gemcitabine;Cisplatin
48 Phase III Randomized, Placebo-controlled Clinical Trial of Hepcortespenlisimut-L (Hepko-V5) Versus Placebo in Patients With Advanced Hepatocellular Carcinoma (HCC) Recruiting NCT02232490 Phase 3
49 A Randomized Non-inferiority Trial Evaluating the Length of Treatment With PD-1/PD-L1 Inhibitors in Patients With Advanced Solid Tumors Recruiting NCT04157985 Phase 3 Continue PD-1/PD-L1 Inhibitors treatment
50 A Phase III Open-Label, Multi-Centre, Randomized Study Comparing NUC-1031 Plus Cisplatin to Gemcitabine Plus Cisplatin in Patients With Previously Untreated Locally Advanced or Metastatic Biliary Tract Cancer Recruiting NCT04163900 Phase 3 NUC-1031;Gemcitabine;Cisplatin

Search NIH Clinical Center for Cholangiocarcinoma

Cell-based therapeutics:

LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Cholangiocarcinoma cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Cholangiocarcinoma:
Tumor infiltrating lymphocytes (TILs) for solid tumors
Embryonic/Adult Cultured Cells Related to Cholangiocarcinoma:
Tumor infiltrating lymphocytes PMIDs: 24329789 19342963 21498393 22996367 19304471 22555974 15800326 23650429 21325070 8170938 12242449 24218514 23904171

Genetic Tests for Cholangiocarcinoma

Genetic tests related to Cholangiocarcinoma:

# Genetic test Affiliating Genes
1 Cholangiocarcinoma, Susceptibility to 29
2 Cholangiocarcinoma 29

Anatomical Context for Cholangiocarcinoma

MalaCards organs/tissues related to Cholangiocarcinoma:

Liver, Lymph Node, T Cells, Skin, Pancreas, Colon, Breast

Publications for Cholangiocarcinoma

Articles related to Cholangiocarcinoma:

(show top 50) (show all 12263)
# Title Authors PMID Year
Necroptosis microenvironment directs lineage commitment in liver cancer. 56 61
30209397 2018
The role of microRNA expression pattern in human intrahepatic cholangiocarcinoma. 46 54 61
19070389 2009
Exome sequencing identifies frequent inactivating mutations in BAP1, ARID1A and PBRM1 in intrahepatic cholangiocarcinomas. 56
24185509 2013
Exome sequencing identifies distinct mutational patterns in liver fluke-related and non-infection-related bile duct cancers. 56
24185513 2013
miR-373 negatively regulates methyl-CpG-binding domain protein 2 (MBD2) in hilar cholangiocarcinoma. 61 46
21086164 2011
MicroRNA-dependent regulation of DNA methyltransferase-1 and tumor suppressor gene expression by interleukin-6 in human malignant cholangiocytes. 61 46
20146264 2010
Epigenetic regulation of microRNA-370 by interleukin-6 in malignant human cholangiocytes. 61 46
17621267 2008
Involvement of human micro-RNA in growth and response to chemotherapy in human cholangiocarcinoma cell lines. 61 46
16762633 2006
Alpha-fetoprotein producing cells act as cancer progenitor cells in human cholangiocarcinoma. 61 54
20149523 2010
Secretin inhibits cholangiocarcinoma growth via dysregulation of the cAMP-dependent signaling mechanisms of secretin receptor. 54 61
19904746 2010
The significance of galectin-3 immunohistochemistry, clinical characteristics and liver imaging in differentiating intrahepatic cholangiocarcinoma from adenocarcinoma liver metastasis. 61 54
20524437 2010
Nerve growth factor-beta expression is associated with lymph node metastasis and nerve infiltration in human hilar cholangiocarcinoma. 61 54
20119692 2010
Epidermal growth factor receptor and HER-2/neu status by immunohistochemistry and fluorescence in situ hybridization in adenocarcinomas of the biliary tree and gallbladder. 54 61
20040392 2010
[Detection of lymph node micrometastasis for patients with extrahepatic cholangiocarcinoma and its prognostic significance]. 54 61
20450726 2010
Current research in perineural invasion of cholangiocarcinoma. 54 61
20219134 2010
Significance of mucin expression in pancreatobiliary neoplasms. 61 54
19787286 2010
Involvement of PI3K and ERK1/2 pathways in hepatocyte growth factor-induced cholangiocarcinoma cell invasion. 61 54
20135719 2010
The use of Cytokeratin 19 (CK19) immunohistochemistry in lesions of the pancreas, gastrointestinal tract, and liver. 54 61
19956064 2010
Suppression of galectin-3 expression enhances apoptosis and chemosensitivity in liver fluke-associated cholangiocarcinoma. 54 61
19723119 2009
Expression of MUC1, MUC2, MUC5AC and MUC6 in cholangiocarcinoma: prognostic impact. 61 54
19639217 2009
Endothelin inhibits cholangiocarcinoma growth by a decrease in the vascular endothelial growth factor expression. 54 61
19291182 2009
Expression of KL-6 mucin, a human MUC1 mucin, in intrahepatic cholangiocarcinoma and its potential involvement in tumor cell adhesion and invasion. 54 61
19631667 2009
Monoclonal antibodies against human aspartyl (asparaginyl) beta-hydroxylase developed by DNA immunization. 54 61
19663697 2009
Human cholangiocarcinoma development is associated with dysregulation of opioidergic modulation of cholangiocyte growth. 54 61
18948067 2009
Overexpression of adenovirus-mediated p27kip1 lacking the Jab1-binding region enhances cytotoxicity and inhibits xenografted human cholangiocarcinoma growth. 61 54
19528460 2009
Expression of c-erbB-2 and cyclooxygenase-2 in intrahepatic cholangiocarcinoma. 61 54
19621664 2009
ZD1839 (IRESSA) stabilizes p27Kip1 and enhances radiosensitivity in cholangiocarcinoma cell lines. 61 54
19414361 2009
Detection of serum MMP-7 and MMP-9 in cholangiocarcinoma patients: evaluation of diagnostic accuracy. 54 61
19405942 2009
Resistance of cholangiocarcinoma cells to parthenolide-induced apoptosis by the excretory-secretory products of Clonorchis sinensis. 61 54
19066964 2009
Vandetanib (ZD6474), an inhibitor of VEGFR and EGFR signalling, as a novel molecular-targeted therapy against cholangiocarcinoma. 61 54
19319137 2009
Glypican-3 is a useful diagnostic marker for a component of hepatocellular carcinoma in human liver cancer. 54 61
19212669 2009
Cyclooxygenase-2 is involved in the up-regulation of matrix metalloproteinase-9 in cholangiocarcinoma induced by tumor necrosis factor-alpha. 61 54
19218340 2009
Estrogens stimulate the proliferation of human cholangiocarcinoma by inducing the expression and secretion of vascular endothelial growth factor. 61 54
18395502 2009
Inflammation and tumor progression: a lesson from TNF-alpha-dependent FAK signaling in cholangiocarcinoma. 54 61
19347283 2009
A comprehensive analysis of immunohistochemical studies in intrahepatic cholangiocarcinoma using the survival tree model. 61 54
19262069 2009
Proteomic analysis of human bile from malignant biliary stenosis induced by pancreatic cancer. 61 54
19055478 2009
Role of ErbB family receptor tyrosine kinases in intrahepatic cholangiocarcinoma. 54 61
19084911 2008
[Expression of mucin glycoproteins and cytokeratins in intrahepatic cholangiocarcinoma]. 61 54
19094709 2008
Clinical significance of cell cycle- and apoptosis-related markers in biliary tract cancer: a tissue microarray-based approach revealing a distinctive immunophenotype for intrahepatic and extrahepatic cholangiocarcinomas. 61 54
18854271 2008
Regulation of Wnt/beta-catenin pathway by cPLA2alpha and PPARdelta. 54 61
18636547 2008
[Evaluation of immunohistochemical markers for differential diagnosis of hepatocellular carcinoma from intrahepatic cholangiocarcinoma]. 54 61
19173916 2008
Clinicopathological analysis of colorectal cancer liver metastasis and intrahepatic cholangiocarcinoma: are they just apples and oranges? 61 54
18329969 2008
Bile acids repress E-cadherin through the induction of Snail and increase cancer invasiveness in human hepatobiliary carcinoma. 61 54
18691339 2008
BH3-only protein mimetic obatoclax sensitizes cholangiocarcinoma cells to Apo2L/TRAIL-induced apoptosis. 54 61
18723481 2008
Expression of cell cycle-related molecules in biliary premalignant lesions: biliary intraepithelial neoplasia and biliary intraductal papillary neoplasm. 61 54
18495210 2008
Expression of matrix metalloproteinase 7 is an unfavorable postoperative prognostic factor in cholangiocarcinoma of the perihilar, hilar, and extrahepatic bile ducts. 54 61
18329694 2008
c-Met targeted therapy of cholangiocarcinoma. 54 61
18494048 2008
Aberrant expression of alpha-fetoprotein in intrahepatic cholangiocarcinoma: an exceptional occurrence. 61 54
18417680 2008
[Expression of melanoma antigen-1, 3 genes in human intrahepatic cholangiocarcinoma and its clinical significance]. 61 54
18505125 2008
Clinicopathologic characteristics of intrahepatic cholangiocarcinoma in patients with positive serum a-fetoprotein. 61 54
18407604 2008

Variations for Cholangiocarcinoma

ClinVar genetic disease variations for Cholangiocarcinoma:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 FGFR2-CLIP1 fusionfusion Pathogenic 637959

Cosmic variations for Cholangiocarcinoma:

9 (show top 50) (show all 5411)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM149332377 ZFHX3 biliary tract,bile duct,carcinoma,NS c.3529+1G>A p.? 16:72829778-72829778 9
2 COSM149275977 ZFHX3 biliary tract,bile duct,carcinoma,NS c.6964C>T p.R2322* 16:72795718-72795718 9
3 COSM143040603 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 9
4 COSM101978949 XPO1 biliary tract,bile duct,carcinoma,NS c.338C>T p.T113M 2:61502274-61502274 9
5 COSM101977429 XPO1 biliary tract,bile duct,carcinoma,NS c.729G>T p.E243D 2:61498703-61498703 9
6 COSM101981179 XPO1 biliary tract,bile duct,carcinoma,NS c.273G>T p.W91C 2:61522639-61522639 9
7 COSM90480526 USP20 biliary tract,bile duct,carcinoma,NS c.1378C>G p.L460V 9:129869411-129869411 9
8 COSM85721542 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 9
9 COSM85726810 TSC1 biliary tract,bile duct,carcinoma,NS c.2551C>T p.Q851* 9:132900789-132900789 9
10 COSM86229821 TRIM28 biliary tract,bile duct,carcinoma,NS c.1475G>T p.R492L 19:58549053-58549053 9
11 COSM87921436 TP53 biliary tract,bile duct,carcinoma,NS c.1015G>T p.E339* 17:7670694-7670694 9
12 COSM87897711 TP53 biliary tract,bile duct,carcinoma,NS c.818G>A p.R273H 17:7673802-7673802 9
13 COSM87897745 TP53 biliary tract,bile duct,carcinoma,NS c.524G>A p.R175H 17:7675088-7675088 9
14 COSM88042819 TP53 biliary tract,bile duct,carcinoma,NS c.574C>A p.Q192K 17:7674957-7674957 9
15 COSM87899169 TP53 biliary tract,bile duct,carcinoma,NS c.993+1G>T p.? 17:7673534-7673534 9
16 COSM87918666 TP53 biliary tract,bile duct,carcinoma,NS c.535C>A p.H179N 17:7675077-7675077 9
17 COSM87899497 TP53 biliary tract,bile duct,carcinoma,NS c.586C>T p.R196* 17:7674945-7674945 9
18 COSM88030468 TP53 biliary tract,bile duct,carcinoma,NS c.568C>G p.P190A 17:7674963-7674963 9
19 COSM87906520 TP53 biliary tract,bile duct,carcinoma,NS c.726C>T p.C242= 17:7674237-7674237 9
20 COSM87897752 TP53 biliary tract,bile duct,carcinoma,NS c.731G>A p.G244D 17:7674232-7674232 9
21 COSM87948469 TP53 biliary tract,bile duct,carcinoma,NS c.395A>C p.K132T 17:7675217-7675217 9
22 COSM87898714 TP53 biliary tract,bile duct,carcinoma,NS c.844C>T p.R282W 17:7673776-7673776 9
23 COSM88016238 TP53 biliary tract,bile duct,carcinoma,NS c.702C>G p.Y234* 17:7674261-7674261 9
24 COSM87913576 TP53 biliary tract,bile duct,carcinoma,NS c.536A>T p.H179L 17:7675076-7675076 9
25 COSM87899661 TP53 biliary tract,bile duct,carcinoma,NS c.797G>A p.G266E 17:7673823-7673823 9
26 COSM87900604 TP53 biliary tract,bile duct,carcinoma,NS c.637C>T p.R213* 17:7674894-7674894 9
27 COSM87914097 TP53 biliary tract,bile duct,carcinoma,NS c.808T>C p.F270L 17:7673812-7673812 9
28 COSM87947692 TP53 biliary tract,bile duct,carcinoma,NS c.770T>C p.L257P 17:7674193-7674193 9
29 COSM87933529 TP53 biliary tract,bile duct,carcinoma,NS c.380C>A p.S127Y 17:7675232-7675232 9
30 COSM87902369 TP53 biliary tract,bile duct,carcinoma,NS c.1009C>T p.R337C 17:7670700-7670700 9
31 COSM87922049 TP53 biliary tract,bile duct,carcinoma,NS c.713G>T p.C238F 17:7674250-7674250 9
32 COSM87898588 TP53 biliary tract,bile duct,carcinoma,NS c.711G>A p.M237I 17:7674252-7674252 9
33 COSM87898723 TP53 biliary tract,bile duct,carcinoma,NS c.817C>T p.R273C 17:7673803-7673803 9
34 COSM87906659 TP53 biliary tract,bile duct,carcinoma,NS c.659A>C p.Y220S 17:7674872-7674872 9
35 COSM87911941 TP53 biliary tract,bile duct,carcinoma,NS c.749C>T p.P250L 17:7674214-7674214 9
36 COSM87925339 TP53 biliary tract,bile duct,carcinoma,NS c.863A>G p.N288S 17:7673757-7673757 9
37 COSM87897649 TP53 biliary tract,bile duct,carcinoma,NS c.527G>A p.C176Y 17:7675085-7675085 9
38 COSM87980321 TP53 biliary tract,bile duct,carcinoma,NS c.782+2T>G p.? 17:7674179-7674179 9
39 COSM87908034 TP53 biliary tract,bile duct,carcinoma,NS c.473G>A p.R158H 17:7675139-7675139 9
40 COSM87906417 TP53 biliary tract,bile duct,carcinoma,NS c.730G>A p.G244S 17:7674233-7674233 9
41 COSM87905712 TP53 biliary tract,bile duct,carcinoma,NS c.775G>T p.D259Y 17:7674188-7674188 9
42 COSM88043134 TP53 biliary tract,bile duct,carcinoma,NS c.826G>A p.A276T 17:7673794-7673794 9
43 COSM87901171 TP53 biliary tract,bile duct,carcinoma,NS c.395A>G p.K132R 17:7675217-7675217 9
44 COSM87922154 TP53 biliary tract,bile duct,carcinoma,NS c.817C>A p.R273S 17:7673803-7673803 9
45 COSM88004162 TP53 biliary tract,bile duct,carcinoma,NS c.643A>C p.S215R 17:7674888-7674888 9
46 COSM87912858 TP53 biliary tract,bile duct,carcinoma,NS c.533A>C p.H178P 17:7675079-7675079 9
47 COSM87897737 TP53 biliary tract,bile duct,carcinoma,NS c.536A>C p.H179P 17:7675076-7675076 9
48 COSM87898351 TP53 biliary tract,bile duct,carcinoma,NS c.743G>A p.R248Q 17:7674220-7674220 9
49 COSM88009210 TP53 biliary tract,bile duct,carcinoma,NS c.343C>T p.H115Y 17:7676026-7676026 9
50 COSM88428441 TP53 biliary tract,bile duct,carcinoma,NS c.916C>A p.R306= 17:7673704-7673704 9

Copy number variations for Cholangiocarcinoma from CNVD:

# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 111150 17 35097918 35138441 Copy number ERBB2 Cholangiocarcinoma
2 118016 17 7512444 7519279 Copy number P53 Cholangiocarcinoma
3 159441 21 42600000 48129895 Amplification TFF1 Cholangiocarcinoma
4 159442 21 42600000 48129895 Amplification TFF2 Cholangiocarcinoma
5 159443 21 42600000 48129895 Amplification TFF3 Cholangiocarcinoma

Expression for Cholangiocarcinoma

Search GEO for disease gene expression data for Cholangiocarcinoma.

Pathways for Cholangiocarcinoma

Pathways related to Cholangiocarcinoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.51 MIR373 MIR21 MIR200B MIR152 MIR141

GO Terms for Cholangiocarcinoma

Cellular components related to Cholangiocarcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular vesicle GO:1903561 8.8 MIR21 MIR152 MIR148A

Biological processes related to Cholangiocarcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell migration GO:0030336 9.43 MIR320A MIR21 MIR204
2 gene silencing by miRNA GO:0035195 9.36 NEAT1 MIR373 MIR370 MIR320A MIR21 MIR204
3 negative regulation of blood vessel endothelial cell migration GO:0043537 9.33 MIR204 MIR200B MIR152
4 miRNA mediated inhibition of translation GO:0035278 9.26 MIR21 MIR204 MIR200B MIR148A

Molecular functions related to Cholangiocarcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.23 MIR373 MIR320A MIR21 MIR204 MIR200B MIR152
2 mRNA 3'-UTR binding GO:0003730 9.13 MIR21 MIR200B MIR152

Sources for Cholangiocarcinoma

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
54 Novoseek
57 OMIM via Orphanet
61 PubMed
70 Tocris
72 UMLS via Orphanet
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