ORPHA:97282
VIPoma
Also known as: Diarrheogenic islet cell tumor · Pancreatic cholera · VIP-secreting tumor · Verner-Morrison syndrome · WDHA syndrome · Watery diarrhea-hypokalemia-achlorhydria syndrome
Publications
1,865
Trials
7
Interventional, condition-specific
Researchers
1,129
Distinct authors in sample
Gene link
—
Readiness
4/6
Stages with a signal
Clinical definition (Orphanet)
VIPoma is an extremely rare type of pancreatic neuroendocrine tumor that secretes vasoactive intestinal polypeptide (VIP) leading to the manifestations of watery diarrhea, hypokalemia and achlorhydia or hypochhlorhydia (known as WDHA syndrome).
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0019960
- MeSH:D003969
- UMLS:C0011993
- NCIT:C26749
Additional Mondo synonyms (15)
Diarrheogenic islet cell tumour · VIP producing neoplasm · VIP- secreting neoplasm · VIP- secreting tumor · VIP- secreting tumour · VIP-producing NET · VIP-producing neuroendocrine tumor · VIP-producing neuroendocrine tumour · VIP-secreting tumour · malignant vasoactive intestinal peptide-secreting tumour · vasoactive intestinal peptide producing neoplasm · vasoactive intestinal peptide producing tumor · vasoactive intestinal peptide producing tumour · vasoactive intestinal peptide secreting neoplasm · vasoactive intestinal peptide-secreting 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.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
1,865 matched papers (685 in last 10 years) Source
- Phenotype characterisedPresent
41 HPO annotations (e.g. Hypokalemia; Secretory diarrhea; Diabetes mellitus) Source
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationPartial
1 FDA designation (none yet with FDA orphan-indication approval) — e.g. Octreotide Source
- Interventional trialPresent
7 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)
41
Associated phenotypes · MONDO:0019960
- Hypokalemia
- Secretory diarrhea
- Diabetes mellitus
- Dehydration
- Nausea and vomiting
Showing 5 of 41 — 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)
1
Designation · no FDA orphan-indication approval yet
- FDA Octreotide (Sandostatin LAR)Diarrhea Vipoma Tumors · 1998-08-24
Sources: FDA OOPD · EMA orphan designations
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
2 associated chemicals · 46 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.
- Fluorouracil · therapeutic
- Lomustine · therapeutic
Pathways: Cytokine-cytokine receptor interaction; PI3K-Akt signaling pathway; Toll-like receptor signaling pathway; NOD-like receptor signaling pathway; RIG-I-like receptor signaling pathway; Cytosolic DNA-sensing pathway; Jak-STAT signaling pathway; Natural killer cell mediated cytotoxicity
Literature
Is anyone studying this?
1,865
1,865 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.
1,865 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).
685 in the last 10 years · low confidence
Phrase hits: 1,865 · MeSH hits: 46
Who's working on it?
1,129
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.
- 01Bala MM5 papers · 2024
Jagiellonian University Medical College, Chair of Epidemiology and Preventive Medicine, Department of Hygiene and Dietetics; Systematic Reviews Unit, Krakow, Poland.
Papers in Europe PMC - 02Hofland J5 papers · 2026
Department of Internal Medicine, Section of Endocrinology, ENETS Center of Excellence, Erasmus MC Cancer Institute, Rotterdam, the Netherlands.
Papers in Europe PMC - 03Mitus JW5 papers · 2024
The Maria Sklodowska-Curie Institute, Oncology Center, Krakow Branch; Department of Anatomy, Jagiellonian University Medical College, Department of Surgical Oncology, ul. Garncarska 11, Krakow, Poland, 31-115.
Papers in Europe PMC - 04Pedziwiatr M5 papers · 2024
Jagiellonian University Medical College, 2nd Department of General Surgery, Jakubowskiego Street 2, Krakow, Malopolska, Poland, 30-688.
Papers in Europe PMC - 05Storman D5 papers · 2024
Jagiellonian University Medical College, Chair of Epidemiology and Preventive Medicine, Department of Hygiene and Dietetics; Systematic Reviews Unit, Krakow, Poland.
Papers in Europe PMC - 06Swierz MJ5 papers · 2024
Jagiellonian University Medical College, Chair of Epidemiology and Preventive Medicine, Department of Hygiene and Dietetics; Systematic Reviews Unit, Krakow, Poland.
Papers in Europe PMC - 07Wolff R5 papers · 2024
Kleijnen Systematic Reviews Ltd, Unit 6, Escrick Business Park, Riccall Road, Escrick, York, UK, YO19 6FD.
Papers in Europe PMC - 08Bartsch DK4 papers · 2026
Department of Visceral, Thoracic and Vascular Surgery, Phillips-University Marburg and University Hospital Gießen Marburg GmbH, Marburg, Germany.
Papers in Europe PMC - 09Ito T4 papers · 2026
Taichi Nakamura, Robert T Jensen, Cell Biology Section, NIDDK, National Institutes of Health, Bethesda, Maryland, MD 20814, United States.
Papers in Europe PMC - 10Kleijnen J4 papers · 2021
School for Public Health and Primary Care (CAPHRI), Maastricht University, Maastricht, Netherlands, 6200 MD.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
7
interventional trials for this specific condition
7 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 11 September 2026
7 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 90.9th percentile).
low confidence · 90.9th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
7 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
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.
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 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · 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).
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 VIPoma — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This 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.
"VIPoma" OR "Diarrheogenic islet cell tumor" OR "Pancreatic cholera" OR "VIP-secreting tumor" OR "Verner-Morrison syndrome" OR "WDHA syndrome" OR "Watery diarrhea-hypokalemia-achlorhydria syndrome" OR "Diarrheogenic islet cell tumour" OR "VIP producing neoplasm" OR "VIP- secreting neoplasm" OR "VIP- secreting tumor" OR "VIP- secreting tumour" OR "VIP-producing NET" OR "VIP-producing neuroendocrine tumor" OR "VIP-producing neuroendocrine tumour" OR "VIP-secreting tumour" OR "malignant vasoactive intestinal peptide-secreting tumour" OR "vasoactive intestinal peptide producing neoplasm" OR "vasoactive intestinal peptide producing tumor" OR "vasoactive intestinal peptide producing tumour" OR "vasoactive intestinal peptide secreting neoplasm" OR "vasoactive intestinal peptide-secreting tumour"
MeSH descriptor terms unioned into the query: Vipoma
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"VIPoma" OR "Diarrheogenic islet cell tumor" OR "Pancreatic cholera" OR "VIP-secreting tumor" OR "Verner-Morrison syndrome" OR "WDHA syndrome" OR "Watery diarrhea-hypokalemia-achlorhydria syndrome" OR "Diarrheogenic islet cell tumour" OR "VIP producing neoplasm" OR "VIP- secreting neoplasm" OR "VIP- secreting tumor" OR "VIP- secreting tumour" OR "VIP-producing NET" OR "VIP-producing neuroendocrine tumor" OR "VIP-producing neuroendocrine tumour" OR "VIP-secreting tumour" OR "malignant vasoactive intestinal peptide-secreting tumour" OR "vasoactive intestinal peptide producing neoplasm" OR "vasoactive intestinal peptide producing tumor" OR "vasoactive intestinal peptide producing tumour" OR "vasoactive intestinal peptide secreting neoplasm" OR "vasoactive intestinal peptide-secreting tumour"
Interventional trials matched via: both, 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: 7 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is short or not clearly distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
- Publication count (1865) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity
Ingested 2026-07-27T05:05:31.694Z