Zollinger-Ellison syndrome
diseaseOn this page
Also known as pancreatic ulcerogenic tumor syndromepancreatic ulcerogenic tumour syndromeZ E syndromeZESZollinger Ellison syndromeZollinger-Ellison syndrome (disease)
Summary
Zollinger-Ellison syndrome (MONDO:0019610) is a disease and 11 clinical trials. Top therapeutic interventions include lansoprazole, omeprazole, and pantoprazole. A subtype of gastrin secretion abnormality — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
- Phenotypes (HPO): 34
- Clinical trials: 11
Clinical features
Epidemiology
Prevalence records
6 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.125 | Worldwide | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.15 | Europe | Validated |
| Point prevalence | 1-9 / 100 000 | Europe | Validated | |
| Annual incidence | 1-9 / 1 000 000 | 0.15 | Denmark | Validated |
| Annual incidence | <1 / 1 000 000 | 0.05 | Ireland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.3 | Switzerland | Not yet validated |
Signs & symptoms
Clinical features (HPO)
34 HPO clinical features (Orphanet curated; top 34 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0002044 | Zollinger-Ellison syndrome | Obligate (100%) |
| HP:0100634 | Neuroendocrine neoplasm | Obligate (100%) |
| HP:0002014 | Diarrhea | Very frequent (80-99%) |
| HP:0002018 | Nausea | Very frequent (80-99%) |
| HP:0002574 | Episodic abdominal pain | Very frequent (80-99%) |
| HP:0002588 | Duodenal ulcer | Very frequent (80-99%) |
| HP:0004398 | Peptic ulcer | Very frequent (80-99%) |
| HP:0100633 | Esophagitis | Very frequent (80-99%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0000843 | Hyperparathyroidism | Occasional (5-29%) |
| HP:0000845 | Elevated circulating growth hormone concentration | Occasional (5-29%) |
| HP:0000854 | Thyroid adenoma | Occasional (5-29%) |
| HP:0000952 | Jaundice | Occasional (5-29%) |
| HP:0001012 | Multiple lipomas | Occasional (5-29%) |
| HP:0002239 | Gastrointestinal hemorrhage | Occasional (5-29%) |
| HP:0002573 | Hematochezia | Occasional (5-29%) |
| HP:0002893 | Pituitary adenoma | Occasional (5-29%) |
| HP:0003072 | Hypercalcemia | Occasional (5-29%) |
| HP:0003118 | Increased circulating cortisol level | Occasional (5-29%) |
| HP:0003165 | Elevated circulating parathyroid hormone level | Occasional (5-29%) |
| HP:0005214 | Intestinal obstruction | Occasional (5-29%) |
| HP:0006767 | Pituitary prolactin cell adenoma | Occasional (5-29%) |
| HP:0008208 | Parathyroid hyperplasia | Occasional (5-29%) |
| HP:0008256 | Adrenocortical adenoma | Occasional (5-29%) |
| HP:0008291 | Pituitary corticotropic cell adenoma | Occasional (5-29%) |
| HP:0010783 | Erythema | Occasional (5-29%) |
| HP:0011760 | Pituitary growth hormone cell adenoma | Occasional (5-29%) |
| HP:0011761 | Pituitary null cell adenoma | Occasional (5-29%) |
| HP:0012030 | Increased urinary cortisol level | Occasional (5-29%) |
| HP:0012032 | Lipoma | Occasional (5-29%) |
| HP:0012334 | Extrahepatic cholestasis | Occasional (5-29%) |
| HP:0030404 | Glucagonoma | Occasional (5-29%) |
| HP:0030688 | Increased glucagon level | Occasional (5-29%) |
| HP:0006744 | Adrenocortical carcinoma | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | Zollinger-Ellison syndrome |
| Mondo ID | MONDO:0019610 |
| EFO | EFO:0007549 |
| MeSH | D015043 |
| Orphanet | 913 |
| DOID | DOID:0050782 |
| ICD-11 | 375645550 |
| NCIT | C3453 |
| SNOMED CT | 302824004, 53132006 |
| UMLS | C0043515 |
| MedGen | 53129 |
| GARD | 0007918 |
| MedDRA | 10017852 |
| NORD | 1877 |
| Is cancer (heuristic) | no |
Also known as: pancreatic ulcerogenic tumor syndrome · pancreatic ulcerogenic tumour syndrome · Z E syndrome · ZES · Zollinger Ellison syndrome · Zollinger-Ellison syndrome · Zollinger-Ellison syndrome (disease)
Data availability: 1 HPO phenotype.
Disease family
This is a subtype of gastrin secretion abnormality. 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 › pancreas disorder › endocrine pancreas disorder › gastrin secretion abnormality › Zollinger-Ellison syndrome
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
No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Interferon, Omeprazole, Pantoprazole.
Clinical trials & evidence
Clinical trials
Clinical trials: 11.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 4 |
| Not specified | 3 |
| PHASE3 | 2 |
| PHASE4 | 1 |
| PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00204373 | PHASE4 | COMPLETED | Treatment of Zollinger-Ellison Syndrome With Prevacid |
| NCT00079833 | PHASE3 | COMPLETED | Esomeprazole In Patients With Gastric Acid Hypersecretory States Including Idiopathic Hypersecretion and Zollinger-Ellison Syndrome |
| NCT02153398 | PHASE3 | COMPLETED | A Phase I/III Study of D961H 10 mg and 20 mg in Japanese Paediatric Patients With Gastrointestinal Acid Related Diseases |
| NCT00001165 | PHASE2 | COMPLETED | Combination Chemotherapy in Patients With Zollinger-Ellison Syndrome and Tumors of the Pancreas |
| NCT00001191 | PHASE2 | COMPLETED | The Use of Oral Omeprazole and Intravenous Pantoprazole in Patients With Hypersecretion of Gastric Acid |
| NCT00001228 | PHASE2 | COMPLETED | Interferon and Octreotide to Treat Zollinger-Ellison Syndrome and Advanced Non-B Islet Cell Cancer |
| NCT02454075 | PHASE2 | TERMINATED | YF476 and Type II Gastric Carcinoids |
| NCT02831179 | PHASE1 | WITHDRAWN | Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor |
| NCT00001240 | Not specified | COMPLETED | Evaluating Patients With Abnormal Levels of Gastric Acid |
| NCT00001241 | Not specified | COMPLETED | Treatment of Zollinger-Ellison Syndrome |
| NCT00001254 | Not specified | COMPLETED | Evaluating Pancreatic Tumors in Patients With Zollinger-Ellison Syndrome |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| LANSOPRAZOLE | 4 | 1 |
| OMEPRAZOLE | 4 | 1 |
| PANTOPRAZOLE | 4 | 1 |
| VELIPARIB | 3 | 2 |
| INTERFERON | 3 | 1 |
| NETAZEPIDE | 2 | 1 |
Related Atlas pages
- Drugs: Lansoprazole, Omeprazole, Pantoprazole, Veliparib, Interferon