ORPHA:97280
Glucagonoma
Also known as: Glucagonoma syndrome
Clinical definition (Orphanet)
Glucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor (PNET) that hypersecretes glucagon, leading to a syndrome comprised of necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.
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.
Is anyone studying this?
2,487
2,487 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=183) is 38.
2,487 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).
882 in the last 10 years · low confidence
Is a treatment being tested?
16
trials for this specific condition
16 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 26 July 2026
16 interventional trials — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 88.8th percentile).
low confidence · 88.8th percentile (trials denominator)
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.
Who's working on it?
1,040
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.
- 01Klöppel G6 papers · 2026
Department of Pathology, Technical University Munich, Munich, Germany.
Papers in Europe PMC - 02Faggiano A4 papers · 2025
Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, ENETS Centre of Excellence, Sapienza University of Rome, Rome, Italy.
Papers in Europe PMC - 03Hofland J4 papers · 2026
Department of Internal Medicine, Section of Endocrinology, ENETS Centre of Excellence, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
Papers in Europe PMC - 04Jiang Y4 papers · 2025
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Papers in Europe PMC - 05Panzuto F4 papers · 2026
Department of Medical-Surgical Sciences and Translational Medicine, Sapienza University of Rome, Digestive Disease Unit, Sant'Andrea University Hospital, Rome, Italy.
Papers in Europe PMC - 06Bala MM3 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 - 07Bartsch DK3 papers · 2026
Department of Visceral, Thoracic and Vascular Surgery, Phillips-University Marburg and University Hospital Gießen Marburg GmbH, Marburg, Germany.
Papers in Europe PMC - 08Bevere M3 papers · 2024
ARC-Net Research Center, University of Verona, 37134 Verona, Italy.
Papers in Europe PMC - 09Binquet C3 papers · 2026
INSERM, U1231, EPICAD Team UMR "Lipids, Nutrition, Cancer", Dijon, France.
Papers in Europe PMC - 10Brosens LAA3 papers · 2025
Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
16 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.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
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 below but are not added to the query string.
"Glucagonoma" OR "Glucagonoma syndrome" OR "pancreatic glucagonoma"
MeSH descriptor terms unioned into the query: Glucagonoma
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Glucagonoma" OR "Glucagonoma syndrome" OR "pancreatic glucagonoma"
Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 16 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): MESH:D005935 UMLS:C0017689 NCIT:C95597
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 (2487) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity