Biliary tract neoplasm

disease
On this page

Also known as biliary tract neoplasm (disease)biliary tree neoplasmbiliary tree neoplasm (disease)biliary tree tumorbiliary tree tumourBILIARY_TRACTneoplasm of biliary treetumor of biliary treetumour of biliary treetumour of the extrahepatic bile duct

Summary

Biliary tract neoplasm (MONDO:0005304) is a cancer and 75 clinical trials. Top therapeutic interventions include cisplatin, tremelimumab, and binimetinib. A subtype of biliary tract disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Classification: Cancer
  • Clinical trials: 75

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical namebiliary tract neoplasm
Mondo IDMONDO:0005304
EFOEFO:0003891
DOIDDOID:0050625
SNOMED CT126853008
UMLSC0005426
MedGen14118
Anatomy (UBERON)UBERON:0001173
Is cancer (heuristic)yes

Also known as: biliary tract neoplasm · biliary tract neoplasm (disease) · biliary tree neoplasm · biliary tree neoplasm (disease) · biliary tree tumor · biliary tree tumour · BILIARY_TRACT · neoplasm of biliary tree · tumor of biliary tree · tumour of biliary tree · tumour of the extrahepatic bile duct

Data availability: 1 HPO phenotype.

Disease family

This is a subtype of biliary tract disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorderhepatobiliary disorderbiliary tract disorderbiliary tract neoplasm

Related subtypes (14): bile duct disorder, gallstones, primary biliary cholangitis, bile reflux, postcholecystectomy syndrome, Alagille syndrome, isolated agenesis of gallbladder, cholelithiasis, ketamine-induced biliary dilatation, follicular cholangitis and pancreatitis, idiopathic ductopenia, Caroli syndrome, isolated congenital hepatic fibrosis, Rokitansky-Aschoff sinuses of the gallbladder

Subtypes (2): biliary tract cancer, bile duct neoplasm

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

1 approved, 15 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugDevelopment status
ZanidatamabApproved (phase 4)
BinimetinibPhase 3 (in late-stage trials)
CapecitabinePhase 3 (in late-stage trials)
CisplatinPhase 3 (in late-stage trials)
DurvalumabPhase 3 (in late-stage trials)
EncorafenibPhase 3 (in late-stage trials)
FluorouracilPhase 3 (in late-stage trials)
FutibatinibPhase 3 (in late-stage trials)
GemcitabinePhase 3 (in late-stage trials)
IvosidenibPhase 3 (in late-stage trials)
NeratinibPhase 3 (in late-stage trials)
NiraparibPhase 3 (in late-stage trials)
OxaliplatinPhase 3 (in late-stage trials)
PembrolizumabPhase 3 (in late-stage trials)
TegafurPhase 3 (in late-stage trials)
TrastuzumabPhase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Axitinib, Cediranib, Cetuximab, Envafolimab, Hydroxychloroquine, Ipilimumab, Irinotecan, Lenvatinib, Nivolumab, Panitumumab, Rivoceranib, Sargramostim, Sovatelitide, Tislelizumab, Toripalimab, Trametinib, Tremelimumab.

Clinical trials & evidence

Clinical trials

Clinical trials: 75.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified34
PHASE227
PHASE44
PHASE34
PHASE1/PHASE23
PHASE12
PHASE2/PHASE31

Top trials by phase / activity

NCTPhaseStatusTitle
NCT00980889PHASE4COMPLETEDRCT Steel (Wallstent®) vs Nitinol (Wallflex®) Bile Duct Stent for Palliation of Malignant Obstruction
NCT01315522PHASE4COMPLETEDCovered vs. Uncovered SEMS for Occluded Biliary Metal Stents
NCT01758341PHASE4COMPLETEDRadiofrequency Ablation for Malignant Biliary Obstruction
NCT02943031PHASE4UNKNOWNThe Effect of Individualized Precision Therapy Programs in Patients With BTC
NCT03478488PHASE3ACTIVE_NOT_RECRUITINGProgrammed Death Ligand (PD-L1) Combined With Chemotherapy for Patients With BTC
NCT03875235PHASE3ACTIVE_NOT_RECRUITINGDurvalumab or Placebo in Combination With Gemcitabine/Cisplatin in Patients With 1st Line Advanced Biliary Tract Cancer (TOPAZ-1)
NCT05615818PHASE3RECRUITINGPersonalized Medicine for Advanced Biliary Cancer Patients
NCT00939848PHASE2/PHASE3COMPLETEDCediranib Versus Placebo Plus Cisplatin/Gemcitabine Chemotherapy for Patients With Advanced Biliary Tract Cancers
NCT01731821PHASE3COMPLETEDNonstented Stump-closed vs Duct-to-Mucosa Pancreaticojejunostomy After Pancreaticoduodenectomy
NCT03801083PHASE2RECRUITINGAdoptive Transfer of Tumor Infiltrating Lymphocytes for Biliary Tract Cancers
NCT04308174PHASE2ACTIVE_NOT_RECRUITINGNeoadjuvant Gemcitabine Plus Cisplatin With or Without Durvalumab in Resectable Biliary Tract Cancer
NCT04579380PHASE2ACTIVE_NOT_RECRUITINGBasket Study of Tucatinib and Trastuzumab in Solid Tumors With HER2 Alterations
NCT04910386PHASE2NOT_YET_RECRUITINGMulticenter Phase 2 Study of Envafolimab in Biliary Tract Cancers
NCT04956640PHASE1/PHASE2ACTIVE_NOT_RECRUITINGStudy of LY3537982 in Cancer Patients With a Specific Genetic Mutation (KRAS G12C)
NCT06037655PHASE2NOT_YET_RECRUITINGChemotherapy Combined With Adebrelimab and Mecapegfilgrastim in Neoadjuvant Treatment of Potentially Resectable BTC
NCT06638931PHASE2RECRUITINGAgnostic Therapy in Rare Solid Tumors
NCT07109167PHASE2NOT_YET_RECRUITINGThe Safety and Efficacy of Benmelstobart Injection in Patients With Advanced Biliary Tract Malignant Tumors
NCT07146646PHASE2RECRUITINGTrifluridine/Tipiracil + Oxaliplatin in Participants With Advanced or Metastatic Biliary Tract Cancer
NCT07160283PHASE2NOT_YET_RECRUITINGThe Efficacy and Safety of Trilaciclib in Bone Marrow Protection Before Chemotherapy for Advanced Bile Duct Cancer and Pancreatic Cancer
NCT07269158PHASE1/PHASE2NOT_YET_RECRUITINGA Phase IIb Randomized Clinical Trial of Immune Checkpoint Inhibitor-based Maintenance Therapy in Patients With Advanced Biliary Tract Cancer
NCT00174564PHASE2COMPLETEDEXIBIT: Oxaliplatin in Biliary Tract Cancer
NCT00268840PHASE2COMPLETEDThe Efficacy and Safety of Gemcitabine in Combination With Docetaxel to Treat Pancreatic or Biliary Cancer
NCT00487851PHASE2WITHDRAWNComparing Endoscopic Based Stent Strategy Versus Bypass Surgery in Non-resectable Periampullary Cancer
NCT01853618PHASE1/PHASE2COMPLETEDTremelimumab With Chemoembolization or Ablation for Liver Cancer
NCT02146703PHASE2COMPLETEDGemcitabine and S-1 for Advanced Biliary Tract Cancer
NCT02527824PHASE2UNKNOWNStudy of Oxaliplatin, Irinotecan, and S-1 in Biliary Tract Cancer
NCT02558959PHASE2COMPLETEDSecond-line Irinotecan and Capecitabine Versus Irinotecan for Gemcitabine and Cisplatin Refractory Biliary Tract Cancer
NCT02821754PHASE2COMPLETEDA Pilot Study of Combined Immune Checkpoint Inhibition in Combination With Ablative Therapies in Subjects With Hepatocellular Carcinoma (HCC) or Biliary Tract Carcinomas (BTC)
NCT02829918PHASE2COMPLETEDStudy of Nivolumab in Patients With Advanced Refractory Biliary Tract Cancers
NCT03046862PHASE2UNKNOWNDurvalumab(MEDI4736)/Tremelimumab in Combination With Gemcitabine/Cisplatin in Chemotherapy-naïve Biliary Tract Cancer
NCT03101566PHASE2COMPLETEDStudy of Nivolumab in Combination With Gemcitabine/Cisplatin or Ipilimumab for Patients With Advanced Unresectable Biliary Tract Cancer
NCT03111732PHASE2COMPLETEDPembrolizumab, a Monoclonal Antibody Against PD-1, in Combination With Capecitabine and Oxaliplatin (CAPOX) in People With Advanced Biliary Tract Carcinoma (BTC)
NCT03406299PHASE2COMPLETEDPhase II Randomized Trial of SLOG vs GC in Locally Advanced or Metastatic Biliary Tract Cancer
NCT03427242PHASE2UNKNOWNApatinib for Advanced Biliary Carcinoma
NCT04010071PHASE2UNKNOWNAxitinib Plus Toripalimab as Second-line Treatment in Hepatobiliary Malignant Tumors
NCT04352023PHASE2UNKNOWNAnalgesic Efficacy After Pancreatobiliary Surgery: Intravenous Versus Patient-controlled Epidural
NCT04566133PHASE2TERMINATEDCombination of Trametinib (MEK Inhibitor) and Hydroxychloroquine (HCQ) (Autophagy Inhibitor) in Patients With KRAS Mutation Refractory Bile Tract Carcinoma (BTC).
NCT05190575PHASE2COMPLETEDTST001 in Patients With CLDN18.2 Positive Previously Treated Advanced Biliary Tract Cancer
NCT05935579PHASE2UNKNOWNDurvalumab and Lenvatinib With or Without Chemotherapy in First-Line Treatment of Advanced Biliary Tract Cancer
NCT07614061PHASE1RECRUITINGSafety and Efficacy of CD160-Enhanced Autologous Antigen-Specific T-Cells (BTC-Ag-T) in Advanced Biliary Tract Cancer

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
CISPLATIN48
TREMELIMUMAB43
BINIMETINIB41
CHLOROTRIANISENE41
DURVALUMAB41
ENCORAFENIB41
FUTIBATINIB41
GEMCITABINE41
IVOSIDENIB41
NERATINIB41
NIRAPARIB41
SELUMETINIB41
TEGAFUR41
TIPIRACIL41
TRIFLURIDINE41
TUCATINIB41
ZANIDATAMAB41
CEDIRANIB31
OLOMORASIB31
OSEMITAMAB21
CHEMBL23599301
CHEMBL543395001
CHEMBL572333701
CHEMBL446320901