Carcinoma of the ampulla of vater

Summary

Carcinoma of the ampulla of vater (MONDO:0017590) is a cancer and 9 clinical trials. Top therapeutic interventions include dostarlimab, vandetanib, and surufatinib. A subtype of ampulla of vater cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: 1-9 / 1 000 000 (France) [Orphanet-validated]
• Clinical trials: 9

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.59	France	Validated
Annual incidence	1-9 / 1 000 000	0.57	United Kingdom	Validated
Point prevalence	1-9 / 100 000		Europe	Not yet validated

Identifiers

Disease identifiers

Field	Value
Canonical name	carcinoma of the ampulla of vater
Mondo ID	MONDO:0017590
EFO	EFO:1000079
Orphanet	300557
DOID	DOID:4932
ICD-11	267067189
NCIT	C3908
SNOMED CT	254609000
UMLS	C0262401
MedGen	120461
GARD	0021237
MedDRA	10048853
Anatomy (UBERON)	UBERON:0004913
Is cancer (heuristic)	yes

Also known as: AMPCA · ampulla of Vater carcinoma · ampullary carcinoma · Ampulloma · carcinoma of ampulla of vater · carcinoma of hepatopancreatic ampulla · hepatopancreatic ampulla carcinoma

Data availability: 14 cell lines.

Disease family

This is a subtype of ampulla of vater cancer. 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 disorder › intestinal disorder › intestinal neoplasm › small intestine neoplasm › small intestine cancer › duodenum cancer › ampulla of vater cancer › carcinoma of the ampulla of vater

Subtypes (3): ampulla of vater adenocarcinoma, ampulla of vater squamous cell carcinoma, ampulla of vater small cell neuroendocrine carcinoma

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

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 9.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	6
PHASE2	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06062485	PHASE2	NOT_YET_RECRUITING	Surufatinib Combined With Toripalimab and AG Regiments for First-line Treatment of Unresectable or Relapsing Metastatic Ampullary Carcinoma
NCT06333314	PHASE2	RECRUITING	Dostarlimab for Locally Advanced or Metastatic Cancer (Non-colorectal/Non-endometrial) With Tumor dMMR/MSI
NCT00753675	PHASE2	COMPLETED	Vandetanib Gemcitabine Or Placebo Plus Gemcitabine Or Vandetanib Monotherapy In Advanced Biliary Tract Cancer
NCT07283939	Not specified	RECRUITING	Studying the PAGODA Algorithm for Chemotherapy Dose Changes to Prevent Unplanned Treatment Delays
NCT07328607	Not specified	RECRUITING	Outcomes After Laparoscopic Versus Open Pancreaticoduodenectomy
NCT07446543	Not specified	NOT_YET_RECRUITING	Effect of Negative-Pressure Drainage Versus Pancreatic Duct Stenting for Preventing Pancreatitis After Endoscopic Papillectomy for Duodenal Papillary Tumors
NCT01844245	Not specified	COMPLETED	Endobiliary RFA for Unresectable Malignant Biliary Strictures
NCT02081131	Not specified	COMPLETED	Pancreatic Head and Peri-ampullary Cancer Laparoscopic vs Open Surgical Treatment Trial (PLOT)
NCT02238613	Not specified	UNKNOWN	Radioactive Stents for Treatment of Unresectable Biliary Tract Cancer

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DOSTARLIMAB	4	1
VANDETANIB	4	1
SURUFATINIB	3	1