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ORPHA:100075

Neuroendocrine tumor of stomach

medium confidenceDisorder

Also known as: GNET · Gastric NET · Gastric neuroendocrine tumor · NET of stomach

Publications

1,992

89.4th percentile

Trials

3

Interventional, condition-specific

Researchers

1,251

Distinct authors in sample

Gene link

ATP4A

Limited

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare subtype of neuroendocrine neoplasm, arising from enterochromaffin-like cells in the stomach, with a variable clinical presentation, disease course and prognosis, depending on the disease type and histological grade. Most patients are asymptomatic, with diagnosis usually occurring incidentally during gastroscopy, however, symptoms of dyspepsia, anemia, pain, weight loss and gastrointestinal bleeding can be observed. Association with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I has been reported.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (9)

gastric neuroendocrine neoplasm · neuroendocrine neoplasm of stomach · neuroendocrine neoplasm of the stomach · neuroendocrine tumour of the stomach · stomach NET · stomach neuroendocrine neoplasm · stomach neuroendocrine tumor · stomach neuroendocrine tumor, well differentiated, low or intermediate grade · stomach neuroendocrine tumour

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 identifiedPresent

    Limited — ATP4A

  2. LiteraturePresent

    1,992 matched papers (1,465 in last 10 years) Source

  3. Phenotype characterisedPresent

    32 HPO annotations (e.g. Nausea and vomiting; Anorexia; Intermittent diarrhea) Source

  4. Animal modelNot found

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

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    3 matched on ClinicalTrials.gov (1 recruiting in sample)

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?

Possibly — only limited evidence so far for ATP4A.

GenCC classification: Limited.

Phenotypes (Monarch / HPO)

32

Associated phenotypes · MONDO:0003111

  • Nausea and vomiting
  • Anorexia
  • Intermittent diarrhea
  • Episodic abdominal pain
  • Poor appetite

Showing 5 of 32 — 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)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

1,992

1,992 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

1,992 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).

1,465 in the last 10 years · medium confidence · 89.4th percentile (publications denominator)

Phrase hits: 1,228 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,251

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 · 2026

    Vita e Salute San Raffaele University, Medicine and Surgery, Milan, Italy.

    Papers in Europe PMC
  2. 02
    Annibale B5 papers · 2026

    Department of Medical-Surgical Sciences and Translational Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy.

    Papers in Europe PMC
  3. 03
    Chen J5 papers · 2023

    Department of Gastroenterology, The First Affiliated Hospital Sun Yat-Sen University, Guangzhou 510080, Guangdong Province, China.

    Papers in Europe PMC
  4. 04
    Liu Y5 papers · 2026

    Department of Gastroenterology, the Fifth Medical Center of PLA General Hospital, Beijing, 100071, China.

    Papers in Europe PMC
  5. 05
    Wang Y5 papers · 2026

    Department of Gastric Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou, Guangdong, P. R. China.

    Papers in Europe PMC
  6. 06
    Di Sabatino A4 papers · 2026

    Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy.

    Papers in Europe PMC
  7. 07
    Esposito G4 papers · 2026

    Department of Medical-Surgical Sciences and Translational Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Via di Grottarossa 1035, 00189, Rome, Italy. gianluca.esposito@uniroma1.it.

    Papers in Europe PMC
  8. 08
    Invernizzi P4 papers · 2025

    Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.

    Papers in Europe PMC
  9. 09
    Lenti MV4 papers · 2026

    Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy.

    Papers in Europe PMC
  10. 10
    Li J4 papers · 2023

    Department of Pathology, National Clinical Research Centre for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin's Clinical Research Centre for Cancer, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

3

interventional trials for this specific condition

3 interventional trials matched this specific condition name; 1 currently recruiting in our sample.

Data as of 11 September 2026 · last trial check 28 July 2026

3 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 86.7th percentile).

medium confidence · 86.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

1 observational study matches 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 38 · after dedupe 38 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 38 · dropped 0 · fetched 2026-07-30

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 (38)

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 Neuroendocrine tumor of stomach — 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.

("Neuroendocrine tumor of stomach" OR "Neuroendocrine tumor of the stomach" OR "Gastric NET" OR "Gastric neuroendocrine tumor" OR "NET of stomach" OR "NET of the stomach" OR "gastric neuroendocrine neoplasm" OR "neuroendocrine neoplasm of stomach" OR "neuroendocrine neoplasm of the stomach" OR "neuroendocrine tumour of the stomach" OR "neuroendocrine tumour of stomach" OR "stomach NET" OR "stomach neuroendocrine neoplasm" OR "stomach neuroendocrine tumor" OR "stomach neuroendocrine tumor, well differentiated, low or intermediate grade" OR "stomach neuroendocrine tumour") OR ("ATP4A" OR "ATP4A syndrome" OR "ATP4A-related")

Run this search on Europe PMC

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

"Neuroendocrine tumor of stomach" OR "Neuroendocrine tumor of the stomach" OR "Gastric NET" OR "Gastric neuroendocrine tumor" OR "NET of stomach" OR "NET of the stomach" OR "gastric neuroendocrine neoplasm" OR "neuroendocrine neoplasm of stomach" OR "neuroendocrine neoplasm of the stomach" OR "neuroendocrine tumour of the stomach" OR "neuroendocrine tumour of stomach" OR "stomach NET" OR "stomach neuroendocrine neoplasm" OR "stomach neuroendocrine tumor" OR "stomach neuroendocrine tumor, well differentiated, low or intermediate grade" OR "stomach neuroendocrine tumour"

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: 3 interventional · 1 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

Synonyms dropped by stoplist: GNET

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T07:01:56.344Z