Cholangiocarcinoma (CC)

Categories: Cancer diseases, Liver diseases, Nephrological diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Cholangiocarcinoma

MalaCards integrated aliases for Cholangiocarcinoma:

Name: Cholangiocarcinoma 57 42 58 75 28 53 5 36 16 71
Cholangiocarcinoma, Susceptibility to 57 28
Intrahepatic Cholangiocarcinoma 42 71
Extrahepatic Cholangiocarcinoma 42 71
Adult Primary Cholangiocellular Carcinoma 71
Cholangiocarcinoma of Biliary Tract 42
Perihilar Cholangiocarcinoma 42
Cholangiocellular Carcinoma 42
Distal Cholangiocarcinoma 42
Chlc, Susceptibility to 57
Bile Duct Carcinoma 71
Bile Duct Cancer 58
Chlc 57
Cca 58
Cc 42



1-9/100000 (Worldwide, Worldwide, United States, Europe) >1/1000 (Thailand) 1-9/1000000 (Australia) 58

Age Of Onset:

Adult 58


Orphanet: 58  
Rare hepatic diseases

External Ids:

OMIM® 57 615619
MESH via Orphanet 44 D001650 D018281
ICD10 via Orphanet 32 C22.1 C24.0 C24.8 more
UMLS via Orphanet 72 C0206698 C0740277
Orphanet 58 ORPHA70567
MedGen 40 C3810156
UMLS 71 C0206698 C0280725 C0345905 more

Summaries for Cholangiocarcinoma

MedlinePlus Genetics: 42 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 gallbladder cancer, and has symptoms including nausea and vomiting, constipation and abdominal pain. An important gene associated with Cholangiocarcinoma is MIR21 (MicroRNA 21), and among its related pathways/superpathways is ncRNAs involved in STAT3 signaling in hepatocellular carcinoma. The drugs Celecoxib and Sevoflurane have been mentioned in the context of this disorder. Affiliated tissues include liver, small intestine and lymph node, and related phenotypes are jaundice and biliary tract neoplasm

OMIM®: 57 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) (Updated 08-Dec-2022)

Orphanet: 58 Cholangiocarcinoma (CCA) is a biliary tract cancer (BTC, see this term) originating in the epithelium of the biliary tree, either intra or extra hepatic.

Wikipedia: 75 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 971)
# Related Disease Score Top Affiliating Genes
1 klatskin's tumor 33.0 MIR373 MALAT1
2 gallbladder cancer 31.2 MIR373 MALAT1 H19 AFAP1-AS1
3 cystadenocarcinoma 30.6 MIR21 BRCA2 BRCA1
4 pancreatic ductal adenocarcinoma 30.5 MIR21 MIR148A MIR141 MALAT1 H19 AFAP1-AS1
5 bile duct disease 30.5 MIR21 MIR204 MIR148A
6 thyroid carcinoma 30.5 NEAT1 MALAT1 H19 AFAP1-AS1
7 biliary tract disease 30.3 MIR21 MIR204 MIR148A
8 small cell cancer of the lung 30.1 MIR21 MALAT1 CCAT2
9 lung cancer susceptibility 3 30.1 MIR21 MIR200B MIR141 MALAT1 H19 AFAP1-AS1
10 kidney cancer 30.0 MIR21 MIR204 MIR200B MIR141 MALAT1 H19
11 squamous cell carcinoma 30.0 SOX2-OT PCAT1 NEAT1 MALAT1 H19 CCAT2
12 osteogenic sarcoma 29.9 SOX2-OT PCAT1 NEAT1 MALAT1 HULC H19
13 diffuse large b-cell lymphoma 29.9 MIR320A MIR21 MIR200B HULC
14 pancreatic cancer 29.9 NEAT1 MIR21 MIR148A MALAT1 HULC H19
15 laryngeal squamous cell carcinoma 29.9 NEAT1 MIR21 MALAT1 H19
16 hematologic cancer 29.9 MIR21 MIR204 MIR200B MIR148A MIR141
17 bladder disease 29.8 MIR370 MIR21 MIR204 MIR141
18 bladder cancer 29.8 PCAT1 MIR21 MALAT1 HULC H19 CCAT2
19 stomach disease 29.8 MIR21 MIR204 MIR200B MIR148A MIR141
20 melanoma 29.8 SOX2-OT MIR21 MIR204 MIR200B MIR141 MALAT1
21 gastrointestinal system disease 29.8 MIR21 MIR204 MIR200B MIR148A MIR141
22 thyroid cancer, nonmedullary, 1 29.7 NEAT1 MALAT1 HULC H19
23 hepatocellular carcinoma 29.7 SOX2-OT PCAT1 NEAT1 MIR373 MIR370 MIR320A
24 gastrointestinal system cancer 29.7 MIR21 MIR204 MIR200B MIR148A MIR141 MALAT1
25 larynx cancer 29.7 MIR21 MIR204 MIR148A MIR141
26 endometrial adenocarcinoma 29.7 MIR204 MIR200B MIR152 MALAT1
27 breast-ovarian cancer, familial 1 29.6 BRCA2 BRCA1
28 ovarian cancer 1 29.6 BRCA2 BRCA1
29 glioblastoma 29.5 PCAT1 MIR21 MALAT1 H19 BRCA2 BRCA1
30 lymphatic system disease 29.5 MIR373 MIR21 MIR204 MIR200B MIR148A MIR141
31 retinitis pigmentosa 29.4 SOX2-OT PCAT1 NEAT1 MIR21 MIR204 CCAT2
32 alzheimer disease, familial, 1 29.4 SOX2-OT MIR320A MIR21 MIR204 MIR200B MIR148A
33 breast adenocarcinoma 29.4 H19 BRCA2 BRCA1
34 renal cell carcinoma, nonpapillary 29.4 MIR320A MIR21 MIR204 MIR148A MIR141 MALAT1
35 gastric cancer 29.3 SOX2-OT PCAT1 NEAT1 MIR21 MIR204 MIR141
36 connective tissue disease 29.3 MIR373 MIR21 MIR204 MIR200B MIR152 MIR148A
37 endometrial cancer 29.3 NEAT1 MIR21 MIR200B MIR152 MIR141 H19
38 nasopharyngeal carcinoma 29.3 NEAT1 MIR21 MIR204 MIR152 MIR148A MIR141
39 leukemia, acute myeloid 29.2 MIR320A MIR21 MIR204 MALAT1 BRCA2 BRCA1
40 esophageal cancer 29.2 PCAT1 MIR373 MIR21 MIR204 MIR200B MIR148A
41 ovarian cancer 29.1 SOX2-OT NEAT1 MIR21 MIR200B MIR141 MALAT1
42 colorectal cancer 28.9 SOX2-OT PCAT1 NEAT1 MIR320A MIR21 MIR204
43 oral squamous cell carcinoma 28.7 NEAT1 MIR373 MIR370 MIR21 MIR204 MIR200B
44 breast cancer 28.4 SOX2-OT PTPN3 NEAT1 MIR373 MIR320A MIR21
45 lung cancer 28.3 SOX2-OT PCAT1 NEAT1 MIR373 MIR370 MIR21
46 prostate cancer 28.2 PCAT1 NEAT1 MIR370 MIR320A MIR21 MIR200B
47 intrahepatic cholangiocarcinoma 11.8
48 contractural arachnodactyly, congenital 11.7
49 combined hepatocellular carcinoma and cholangiocarcinoma 11.5
50 bile duct cancer 11.4

Graphical network of the top 20 diseases related to Cholangiocarcinoma:

Diseases related to Cholangiocarcinoma

Symptoms & Phenotypes for Cholangiocarcinoma

Human phenotypes related to Cholangiocarcinoma:

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

Clinical features from OMIM®:

615619 (Updated 08-Dec-2022)

UMLS symptoms related to Cholangiocarcinoma:

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

Drugs & Therapeutics for Cholangiocarcinoma

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

(show top 50) (show all 314)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Celecoxib Approved, Investigational Phase 4 169590-42-5 2662
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
Desflurane Approved Phase 4 57041-67-5 42113
Etomidate Approved Phase 4 33125-97-2 667484
Meperidine Approved Phase 4 57-42-1 4058
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
Levoleucovorin Approved, Experimental, Investigational Phase 4 68538-85-2, 58-05-9, 73951-54-9 149436 6006
Arginine Approved, Investigational, Nutraceutical Phase 4 74-79-3 6322
9 Anti-Inflammatory Agents Phase 4
10 Antirheumatic Agents Phase 4
11 Analgesics, Non-Narcotic Phase 4
12 Platelet Aggregation Inhibitors Phase 4
13 Anesthetics, Inhalation Phase 4
14 Cyclooxygenase Inhibitors Phase 4
15 Cyclooxygenase 2 Inhibitors Phase 4
16 Anti-Inflammatory Agents, Non-Steroidal Phase 4
Bismuth Phase 4 7440-69-9 105143
18 Anesthetics Phase 4
19 Analgesics Phase 4
20 Anti-Anxiety Agents Phase 4
21 Anesthetics, Intravenous Phase 4
22 Anesthetics, General Phase 4
23 Hypnotics and Sedatives Phase 4
24 Analgesics, Opioid Phase 4
25 GABA Modulators Phase 4
26 Narcotics Phase 4
27 Omega 3 Fatty Acid Phase 4
Ulipristal acetate Phase 4 126784-99-4 13559282 130904
29 Calcium, Dietary Phase 4
Calcium Nutraceutical Phase 4 7440-70-2 271
Atezolizumab Approved, Investigational Phase 3 1380723-44-3
Levamisole Approved, Investigational, Vet_approved, Withdrawn Phase 3 14769-73-4 26879
Cemiplimab Approved, Investigational Phase 3 1801342-60-8
Infigratinib Approved, Investigational Phase 3 872511-34-7 53235510
Paclitaxel Approved, Vet_approved Phase 2, Phase 3 33069-62-4 36314
Lenvatinib Approved, Investigational Phase 3 417716-92-8 9823820
Capecitabine Approved, Investigational Phase 3 154361-50-9 60953
Citalopram Approved Phase 3 59729-32-7, 59729-33-8 2771
Dexetimide Withdrawn Phase 3 21888-98-2 30843
40 Liver Extracts Phase 3
41 FOLFIRINOX Phase 2, Phase 3
42 Anti-Bacterial Agents Phase 3
43 Antibiotics, Antitubercular Phase 3
44 Emetics Phase 3
45 Antiparasitic Agents Phase 3
46 Tin Fluorides Phase 2, Phase 3
47 Anthelmintics Phase 3
48 Albumin-Bound Paclitaxel Phase 2, Phase 3
49 Antimitotic Agents Phase 2, Phase 3
50 Tubulin Modulators Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 667)
# Name Status NCT ID Phase Drugs
1 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
2 Effect of Preoperative Biliary Drainage on Postoperative Complications in Resectable Patients With Hilar Cholangiocarcinoma(Klatskin Tumor) Unknown status NCT00797121 Phase 4
3 Early Oral Versus Enteral Nutrition After Pancreatoduodenectomy for Periampullary Tumors: a Prospective, Randomized, Controlled Clinical Trial Unknown status NCT01642875 Phase 4
4 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
5 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)
6 Efficacy and Cost Analysis of Plastic Stent Compare to Metallic Stent in Unresectable Complex Hilar Cholangiocarcinoma; a Randomized Controlled Trial Completed NCT00721175 Phase 4
7 Etomidate With Meperidine vs Midazolam With Meperidine for Sedation During Endodscopic Retrograde Cholangiopancreatogram (ERCP) Completed NCT02027311 Phase 4 Etomidate;Midazolam;Meperidine
8 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
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 Hepatectomy Completed NCT01256047 Phase 4
11 Effects of Preoperative Immunonutrition on Infectious Complication and Th1/Th2 Differentiation in Patients Undergoing Pancreaticoduodenectomy Completed NCT01256034 Phase 4
12 Cholangiocarcinoma Treatment With Radiofrequency Ablation or Photodynamic Therapy: a Randomized Controlled Trial Recruiting NCT05551299 Phase 4 Photosensitizer
13 Recombinant Human Adenovirus Type 5 Combined With Hepatic Artery Infusion Chemotherapy of FOLFOX in Patients With Intrahepatic Mass-forming Cholangiocarcinoma: a Single-site, Single-arm, Prospective Study Not yet recruiting NCT05124002 Phase 4 Recombinant Human Adenovirus Type 5;HAIC of FOLFOX
14 Hepatic Arterial Infusion Chemotherapy of Irinotecan, Oxaliplatin, 5-Fluorouracil and Leucovorin Versus Systemic Chemotherapy of Gemcitabine and Oxaliplatin for Unresectable Intrahepatic Cholangiocarcinoma Unknown status NCT03771846 Phase 3 irinotecan, oxaliplatin , fluorouracil, and leucovorin;gemcitabine and oxaliplatin
15 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
16 Percutaneous Bilateral Versus Unilateral Metal Stent Placement for Hilar Cholangiocarcinoma : A Prospective Open-label Randomized Controlled Trial Unknown status NCT02108145 Phase 2, Phase 3
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 Bortezomib in Treating Patients With Intrahepatic Cholangiocellular Carcinoma Featuring PTEN Deficiency Unknown status NCT03345303 Phase 3 Bortezomib
22 A Randomized, Multicenter, Open-label, Phase 3 Study to Compare the Efficacy and Safety of GEMOX(Gemcitabine/Oxaliplatin) vs XELOX(Xeloda/Oxaliplatin) in Advanced Biliary Tract Carcinoma Unknown status NCT01470443 Phase 3 Oxaliplatin
23 A Prospective, Randomized Trial of Early Enteral Feeding After Pylorus Preserving Pancreatoduodenectomy Unknown status NCT00809081 Phase 3
24 A Phase 3, Multicenter, Randomized, Double-Blind, Placebo-controlled Study of AG-120 in Previously-treated Subjects With Nonresectable or Metastatic Cholangiocarcinoma With an IDH1 Mutation Completed NCT02989857 Phase 3 AG-120;Placebo
25 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
26 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
27 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
28 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 Completed NCT02591030 Phase 2, Phase 3 GEMCIS;mFolfirinox
29 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
30 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
31 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
32 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
33 Phase III Randomized Controlled Trial of a Collaborative Care Intervention to Manage Cancer Related Symptoms in Patients Diagnosed With Hepatobiliary Carcinoma Completed NCT01640522 Phase 3
34 Randomized Study of Early Palliative Care Integrated With Standard Oncology Care Versus Standard Oncology Care Alone in Patients With Incurable Lung or Non-Colorectal Gastrointestinal Malignancies Completed NCT02349412 Phase 3
35 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
36 A Phase 3, Open-Label, Randomized Study of Futibatinib Versus Gemcitabine-Cisplatin Chemotherapy as First-Line Treatment of Patients With Advanced Cholangiocarcinoma Harboring FGFR2 Gene Rearrangements FOENIX-CCA3 Recruiting NCT04093362 Phase 3 TAS-120;Cisplatin/Gemcitabine
37 A Randomized Controlled, Multicenter, Open, Seamless Phase II-III Clinical Trial of PD1 Antibody (Toripalimab), Lenvatinib and GEMOX Neoadjuvant Treatment for Resectable Intrahepatic Cholangiocarcinoma With High-risk Recurrence Factors Recruiting NCT04669496 Phase 2, Phase 3 Neoadjuvant treatment
38 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
39 The Efficacy of Levamisole Hcl in Advanced Intrahepatic Cholangiocarcinoma . A Multicenter, Open, Randomized, Prospective Study Recruiting NCT03940378 Phase 3 Levamisole Hydrochloride;Anlotinib Hydrochloride Capsules
40 Hepatic Arterial Infusion Chemotherapy of Oxaliplatin, 5-Fluorouracil and Leucovorin Versus Systemic Chemotherapy of Gemcitabine and Cisplatin for Unresectable Intrahepatic Cholangiocarcinoma Recruiting NCT04961970 Phase 3 oxaliplatin , fluorouracil, and leucovorin;gemcitabine and cisplatin
41 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
42 Evaluating Length of Treatment With PD-1/PD-L1 Inhibitor in Advanced Solid Tumors Recruiting NCT04157985 Phase 3 Continue PD-1/PD-L1 Inhibitors treatment
43 Adjuvant Chemotherapy With Gemcitabine and Capecitabine Compared to Capecitabine for Biliary Tract Cancer After Curative Resection Recruiting NCT03779035 Phase 3 Gemcitabine;Capecitabine
44 A Phase III Randomized Trial of Gemcitabine, Cisplatin, and Nab-Paclitaxel Versus Gemcitabine and Cisplatin in Newly Diagnosed, Advanced Biliary Tract Cancers Active, not recruiting NCT03768414 Phase 3 Cisplatin;Gemcitabine Hydrochloride;Nab-paclitaxel
45 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 Active, not recruiting NCT03086993 Phase 2, Phase 3 Cisplatin and Gemcitabine
46 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 Active, not recruiting NCT03773302 Phase 3 BGJ398;Gemcitabine;Cisplatin
47 Prospective, Multicenter, Randomized, Controlled Study Evaluating SIR-Spheres Y-90 Resin Microspheres Preceding Cisplatin-gemcitabine (CIS-GEM) Chemotherapy Versus CIS-GEM Chemotherapy Alone as First-line Treatment of Patients With Unresectable Intrahepatic Cholangiocarcinoma Active, not recruiting NCT02807181 Phase 2, Phase 3 Cisplatin-gemcitabine
48 Phase III Study of Radiofrquency Ablation Combined With Cytokine-induced Killer Cells for the Patients With Cholangiocarcinoma Active, not recruiting NCT02482454 Phase 2, Phase 3
49 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 Active, not recruiting NCT04066491 Phase 2, Phase 3 M7824;Placebo;Gemcitabine;Cisplatin
50 Impact of Early Palliative Care on Overall Survival of Patients With Metastatic Upper Gastrointestinal Cancers, Treated With First-line Chemotherapy: a Randomized Phase III Trial Active, not recruiting NCT02853474 Phase 3

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 28
2 Cholangiocarcinoma 28

Anatomical Context for Cholangiocarcinoma

Organs/tissues related to Cholangiocarcinoma:

MalaCards : Liver, Small Intestine, Lymph Node, Skin, T Cells, Pancreas, Colon

Publications for Cholangiocarcinoma

Articles related to Cholangiocarcinoma:

(show top 50) (show all 17709)
# Title Authors PMID Year
Necroptosis microenvironment directs lineage commitment in liver cancer. 62 57
30209397 2018
The role of microRNA expression pattern in human intrahepatic cholangiocarcinoma. 53 62 46
19070389 2009
Exome sequencing identifies frequent inactivating mutations in BAP1, ARID1A and PBRM1 in intrahepatic cholangiocarcinomas. 57
24185509 2013
Exome sequencing identifies distinct mutational patterns in liver fluke-related and non-infection-related bile duct cancers. 57
24185513 2013
miR-373 negatively regulates methyl-CpG-binding domain protein 2 (MBD2) in hilar cholangiocarcinoma. 62 46
21086164 2011
MicroRNA-dependent regulation of DNA methyltransferase-1 and tumor suppressor gene expression by interleukin-6 in human malignant cholangiocytes. 62 46
20146264 2010
Epigenetic regulation of microRNA-370 by interleukin-6 in malignant human cholangiocytes. 62 46
17621267 2008
Involvement of human micro-RNA in growth and response to chemotherapy in human cholangiocarcinoma cell lines. 62 46
16762633 2006
Alpha-fetoprotein producing cells act as cancer progenitor cells in human cholangiocarcinoma. 53 62
20149523 2010
Secretin inhibits cholangiocarcinoma growth via dysregulation of the cAMP-dependent signaling mechanisms of secretin receptor. 53 62
19904746 2010
Nerve growth factor-beta expression is associated with lymph node metastasis and nerve infiltration in human hilar cholangiocarcinoma. 53 62
20119692 2010
The significance of galectin-3 immunohistochemistry, clinical characteristics and liver imaging in differentiating intrahepatic cholangiocarcinoma from adenocarcinoma liver metastasis. 53 62
20524437 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. 53 62
20040392 2010
[Detection of lymph node micrometastasis for patients with extrahepatic cholangiocarcinoma and its prognostic significance]. 53 62
20450726 2010
Current research in perineural invasion of cholangiocarcinoma. 53 62
20219134 2010
Significance of mucin expression in pancreatobiliary neoplasms. 53 62
19787286 2010
Involvement of PI3K and ERK1/2 pathways in hepatocyte growth factor-induced cholangiocarcinoma cell invasion. 53 62
20135719 2010
The use of Cytokeratin 19 (CK19) immunohistochemistry in lesions of the pancreas, gastrointestinal tract, and liver. 53 62
19956064 2010
Suppression of galectin-3 expression enhances apoptosis and chemosensitivity in liver fluke-associated cholangiocarcinoma. 53 62
19723119 2009
Expression of MUC1, MUC2, MUC5AC and MUC6 in cholangiocarcinoma: prognostic impact. 53 62
19639217 2009
Endothelin inhibits cholangiocarcinoma growth by a decrease in the vascular endothelial growth factor expression. 53 62
19291182 2009
Monoclonal antibodies against human aspartyl (asparaginyl) beta-hydroxylase developed by DNA immunization. 53 62
19663697 2009
Expression of KL-6 mucin, a human MUC1 mucin, in intrahepatic cholangiocarcinoma and its potential involvement in tumor cell adhesion and invasion. 53 62
19631667 2009
Human cholangiocarcinoma development is associated with dysregulation of opioidergic modulation of cholangiocyte growth. 53 62
18948067 2009
Overexpression of adenovirus-mediated p27kip1 lacking the Jab1-binding region enhances cytotoxicity and inhibits xenografted human cholangiocarcinoma growth. 53 62
19528460 2009
Expression of c-erbB-2 and cyclooxygenase-2 in intrahepatic cholangiocarcinoma. 53 62
19621664 2009
ZD1839 (IRESSA) stabilizes p27Kip1 and enhances radiosensitivity in cholangiocarcinoma cell lines. 53 62
19414361 2009
Resistance of cholangiocarcinoma cells to parthenolide-induced apoptosis by the excretory-secretory products of Clonorchis sinensis. 53 62
19066964 2009
Detection of serum MMP-7 and MMP-9 in cholangiocarcinoma patients: evaluation of diagnostic accuracy. 53 62
19405942 2009
Vandetanib (ZD6474), an inhibitor of VEGFR and EGFR signalling, as a novel molecular-targeted therapy against cholangiocarcinoma. 53 62
19319137 2009
Cyclooxygenase-2 is involved in the up-regulation of matrix metalloproteinase-9 in cholangiocarcinoma induced by tumor necrosis factor-alpha. 53 62
19218340 2009
Glypican-3 is a useful diagnostic marker for a component of hepatocellular carcinoma in human liver cancer. 53 62
19212669 2009
Estrogens stimulate the proliferation of human cholangiocarcinoma by inducing the expression and secretion of vascular endothelial growth factor. 53 62
18395502 2009
Proteomic analysis of human bile from malignant biliary stenosis induced by pancreatic cancer. 53 62
19055478 2009
A comprehensive analysis of immunohistochemical studies in intrahepatic cholangiocarcinoma using the survival tree model. 53 62
19262069 2009
Inflammation and tumor progression: a lesson from TNF-alpha-dependent FAK signaling in cholangiocarcinoma. 53 62
19347283 2009
Role of ErbB family receptor tyrosine kinases in intrahepatic cholangiocarcinoma. 53 62
19084911 2008
[Expression of mucin glycoproteins and cytokeratins in intrahepatic cholangiocarcinoma]. 53 62
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. 53 62
18854271 2008
Regulation of Wnt/beta-catenin pathway by cPLA2alpha and PPARdelta. 53 62
18636547 2008
Bile acids repress E-cadherin through the induction of Snail and increase cancer invasiveness in human hepatobiliary carcinoma. 53 62
18691339 2008
[Evaluation of immunohistochemical markers for differential diagnosis of hepatocellular carcinoma from intrahepatic cholangiocarcinoma]. 53 62
19173916 2008
Clinicopathological analysis of colorectal cancer liver metastasis and intrahepatic cholangiocarcinoma: are they just apples and oranges? 53 62
18329969 2008
Expression of cell cycle-related molecules in biliary premalignant lesions: biliary intraepithelial neoplasia and biliary intraductal papillary neoplasm. 53 62
18495210 2008
BH3-only protein mimetic obatoclax sensitizes cholangiocarcinoma cells to Apo2L/TRAIL-induced apoptosis. 53 62
18723481 2008
c-Met targeted therapy of cholangiocarcinoma. 53 62
18494048 2008
Expression of matrix metalloproteinase 7 is an unfavorable postoperative prognostic factor in cholangiocarcinoma of the perihilar, hilar, and extrahepatic bile ducts. 53 62
18329694 2008
A novel "patient-like" model of cholangiocarcinoma progression based on bile duct inoculation of tumorigenic rat cholangiocyte cell lines. 53 62
18081149 2008
Clinicopathologic characteristics of intrahepatic cholangiocarcinoma in patients with positive serum a-fetoprotein. 53 62
18407604 2008
Aberrant expression of alpha-fetoprotein in intrahepatic cholangiocarcinoma: an exceptional occurrence. 53 62
18417680 2008

Variations for Cholangiocarcinoma

Cosmic variations for Cholangiocarcinoma:

8 (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 COSM102048118 ZFHX3 biliary tract,bile duct,carcinoma,NS c.787+1G>A p.? 16:72829778-72829778 10
2 COSM87276533 ZFHX3 biliary tract,bile duct,carcinoma,NS c.6964C>T p.R2322* 16:72795718-72795718 10
3 COSM149332377 ZFHX3 biliary tract,bile duct,carcinoma,NS c.3529+1G>A p.? 16:72829778-72829778 10
4 COSM87296816 ZFHX3 biliary tract,bile duct,carcinoma,NS c.3529+1G>A p.? 16:72829778-72829778 10
5 COSM102025189 ZFHX3 biliary tract,bile duct,carcinoma,NS c.4222C>T p.R1408* 16:72795718-72795718 10
6 COSM149275977 ZFHX3 biliary tract,bile duct,carcinoma,NS c.6964C>T p.R2322* 16:72795718-72795718 10
7 COSM90947216 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
8 COSM127997056 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
9 COSM145027300 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
10 COSM128451074 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
11 COSM126972057 YAP1 biliary tract,bile duct,carcinoma,NS c.-101C>G p.? 11:102114256-102114256 10
12 COSM85236535 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
13 COSM143040603 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
14 COSM133273136 YAP1 biliary tract,bile duct,carcinoma,NS c.434C>G p.T145S 11:102114256-102114256 10
15 COSM104236735 XPO1 biliary tract,bile duct,carcinoma,NS c.729G>T p.E243D 2:61498703-61498703 10
16 COSM104240798 XPO1 biliary tract,bile duct,carcinoma,NS c.273G>T p.W91C 2:61522639-61522639 10
17 COSM101978949 XPO1 biliary tract,bile duct,carcinoma,NS c.338C>T p.T113M 2:61502274-61502274 10
18 COSM101977429 XPO1 biliary tract,bile duct,carcinoma,NS c.729G>T p.E243D 2:61498703-61498703 10
19 COSM104888049 XPO1 biliary tract,bile duct,carcinoma,NS c.273G>T p.W91C 2:61522639-61522639 10
20 COSM104238660 XPO1 biliary tract,bile duct,carcinoma,NS c.338C>T p.T113M 2:61502274-61502274 10
21 COSM104883338 XPO1 biliary tract,bile duct,carcinoma,NS c.729G>T p.E243D 2:61498703-61498703 10
22 COSM101981179 XPO1 biliary tract,bile duct,carcinoma,NS c.273G>T p.W91C 2:61522639-61522639 10
23 COSM104885218 XPO1 biliary tract,bile duct,carcinoma,NS c.338C>T p.T113M 2:61502274-61502274 10
24 COSM90480526 USP20 biliary tract,bile duct,carcinoma,NS c.1378C>G p.L460V 9:129869411-129869411 10
25 COSM94050935 USP20 biliary tract,bile duct,carcinoma,NS c.1378C>G p.L460V 9:129869411-129869411 10
26 COSM93928913 USP20 biliary tract,bile duct,carcinoma,NS c.1378C>G p.L460V 9:129869411-129869411 10
27 COSM116185755 UQCC5 biliary tract,bile duct,carcinoma,NS c.152-14931A>G p.? 3:52564087-52564087 10
28 COSM116191591 UQCC5 biliary tract,bile duct,carcinoma,NS c.152-14926G>T p.? 3:52564092-52564092 10
29 COSM116185319 UQCC5 biliary tract,bile duct,carcinoma,NS c.151+13843G>T p.? 3:52550749-52550749 10
30 COSM116188562 UQCC5 biliary tract,bile duct,carcinoma,NS c.151+13571A>T p.? 3:52550477-52550477 10
31 COSM116189844 UQCC5 biliary tract,bile duct,carcinoma,NS c.151+13653G>A p.? 3:52550559-52550559 10
32 COSM116189058 UQCC5 biliary tract,bile duct,carcinoma,NS c.152-15628C>A p.? 3:52563390-52563390 10
33 COSM148042694 TSC1 biliary tract,bile duct,carcinoma,NS c.2533C>T p.Q845* 9:132900789-132900789 10
34 COSM150524491 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
35 COSM111042252 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
36 COSM133088909 TSC1 biliary tract,bile duct,carcinoma,NS c.584+1G>A p.? 9:132921362-132921362 10
37 COSM151875501 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
38 COSM150540889 TSC1 biliary tract,bile duct,carcinoma,NS c.2398C>T p.Q800* 9:132900789-132900789 10
39 COSM85721542 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
40 COSM150681020 TSC1 biliary tract,bile duct,carcinoma,NS c.2536C>T p.Q846* 9:132900789-132900789 10
41 COSM149041137 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
42 COSM150537849 TSC1 biliary tract,bile duct,carcinoma,NS c.584+1G>A p.? 9:132921362-132921362 10
43 COSM102542620 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
44 COSM151876766 TSC1 biliary tract,bile duct,carcinoma,NS c.2551C>T p.Q851* 9:132900789-132900789 10
45 COSM85726810 TSC1 biliary tract,bile duct,carcinoma,NS c.2551C>T p.Q851* 9:132900789-132900789 10
46 COSM148036830 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
47 COSM149045955 TSC1 biliary tract,bile duct,carcinoma,NS c.2548C>T p.Q850* 9:132900789-132900789 10
48 COSM133094049 TSC1 biliary tract,bile duct,carcinoma,NS c.2398C>T p.Q800* 9:132900789-132900789 10
49 COSM151896126 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10
50 COSM148127663 TSC1 biliary tract,bile duct,carcinoma,NS c.737+1G>A p.? 9:132921362-132921362 10

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 TP53 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 9.88 MIR21 MIR200B

GO Terms for Cholangiocarcinoma

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

# Name GO ID Score Top Affiliating Genes
1 RISC complex GO:0016442 9.6 NEAT1 MIR370 MIR320A MIR21 MIR204 MIR200B
2 nuclear ubiquitin ligase complex GO:0000152 9.26 BRCA2 BRCA1

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

# Name GO ID Score Top Affiliating Genes
1 miRNA-mediated gene silencing GO:0035195 9.58 NEAT1 MIR373 MIR370 MIR320A MIR21 MIR204
2 chordate embryonic development GO:0043009 9.5 BRCA2 BRCA1
3 negative regulation of blood vessel endothelial cell migration GO:0043537 9.43 MIR204 MIR200B MIR152
4 miRNA-mediated gene silencing by inhibition of translation GO:0035278 9.26 MIR21 MIR204 MIR200B MIR148A
5 negative regulation of superoxide dismutase activity GO:1901670 9.16 MIR21 H19

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

# Name GO ID Score Top Affiliating Genes
1 mRNA 3'-UTR binding GO:0003730 9.35 MIR21 MIR204 MIR200B MIR152 MIR141
2 mRNA base-pairing translational repressor activity GO:1903231 9.23 MIR373 MIR320A MIR21 MIR204 MIR200B MIR152

Sources for Cholangiocarcinoma

8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
27 GO
28 GTR
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
36 LifeMap
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
70 Tocris
72 UMLS via Orphanet
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