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ORPHA:913

Zollinger-Ellison syndrome

high confidenceDisorder

Also known as: Gastrinoma

Publications

8,755

91th percentile

Trials

23

Interventional, condition-specific

Researchers

1,020

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare neuroendocrine neoplasm (NEN) characterized by severe peptic disease (ulcers/esophageal disease) caused by hypergastrinemia secondary to a gastrin-secreting NEN (gastrinoma) resulting in increased gastric acid secretion.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (2)

Zollinger Ellison syndrome · Zollinger-Ellison syndrome (disease)

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

4/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    8,755 matched papers (2,326 in last 10 years) Source

  3. Phenotype characterisedPresent

    34 HPO annotations (e.g. Zollinger-Ellison syndrome; Neuroendocrine neoplasm; Peptic ulcer) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPartial

    2 FDA designations (none yet with FDA orphan-indication approval) — e.g. Synthetic porcine secretin Source

  6. Interventional trialPresent

    23 matched on ClinicalTrials.gov

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

34

Associated phenotypes · MONDO:0019610

  • Zollinger-Ellison syndrome
  • Neuroendocrine neoplasm
  • Peptic ulcer
  • Gastrointestinal hemorrhage
  • Increased circulating cortisol level

Showing 5 of 34 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

2

Designations · no FDA orphan-indication approval yet

  • FDA Synthetic porcine secretin (Secreflo)Gastrinoma Zollinger-Ellison syndrome · 1999-06-18
  • FDA Synthetic human secretin (Chirostim)Gastrinoma Zollinger-Ellison syndrome · 1999-06-16

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

15

Drugs / clinical candidates · MONDO_0019610

CTD chemicals (MyDisease.info)

5 associated chemicals. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Cimetidine · therapeutic
  • Histamine H2 Antagonists · therapeutic
  • Omeprazole · therapeutic
  • Pantoprazole · therapeutic
  • Ranitidine · therapeutic

MyDisease.info · MONDO:0019610

Literature

Is anyone studying this?

8,755

8,755 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

8,755 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

2,326 in the last 10 years · high confidence · 91th percentile (publications denominator)

Phrase hits: 8,755 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,020

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Massironi S6 papers · 2025

    Division of Gastroenterology Fondazione, IRCCS San Gerardo dei Tintori, Monza, Italia. sara.massironi@libero.it.

    Papers in Europe PMC
  2. 02
    Ito T5 papers · 2025

    Neuroendocrine Tumor Centre, Fukuoka Sanno Hospital, International University of Health and Welfare, 3-6-45 Momochihama, Sawara-Ku, Fukuoka 814-0001, Japan.

    Papers in Europe PMC
  3. 03
    Jensen RT5 papers · 2025

    Digestive Diseases Branch, NIDDK, NIH, Bethesda, MD 20892-1804, USA.

    Papers in Europe PMC
  4. 04
    Cavalcoli F4 papers · 2024

    Gastroenterology and Digestive Endoscopy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy.

    Papers in Europe PMC
  5. 05
    Elvevi A4 papers · 2024

    Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza 20900, Italy.

    Papers in Europe PMC
  6. 06
    Invernizzi P4 papers · 2023

    Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza 20900, Italy.

    Papers in Europe PMC
  7. 07
    Ramos-Alvarez I4 papers · 2025

    Digestive Diseases Branch, NIDDK, NIH, Bethesda, MD 20892-1804, USA.

    Papers in Europe PMC
  8. 08
    Bartsch DK3 papers · 2024

    Department of Surgery, Philipps-University, 35041 Marburg, Germany.

    Papers in Europe PMC
  9. 09
    Blau JE3 papers · 2025

    Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.

    Papers in Europe PMC
  10. 10
    Kasajima A3 papers · 2026

    Department of Pathology, TUM School of Medicine and Health, Technical University Munich, Trogerstr. 18, 81675, Munich, Germany. atsuko.kasajima@tum.de.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

23

interventional trials for this specific condition

23 interventional trials matched this specific condition name; none in our sample are currently recruiting.

Data as of 11 September 2026

23 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 95.3th percentile).

high confidence · 95.3th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

23 interventional trials matched after quoted-phrase search and title/condition post-filter.

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Observational and natural-history studies

5 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

None of the matched observational studies is currently listed as recruiting.

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 9 · after dedupe 9 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 9 · dropped 0 · fetched 2026-07-29

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (9)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Zollinger-Ellison syndrome — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Zollinger-Ellison syndrome" OR "Gastrinoma" OR "Zollinger Ellison syndrome" OR "Zollinger-Ellison syndrome (disease)"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Zollinger-Ellison syndrome" OR "Gastrinoma" OR "Zollinger Ellison syndrome" OR "Zollinger-Ellison syndrome (disease)"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 23 interventional · 5 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T15:54:17.131Z