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glucagonoma - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

glucagonoma

MONDO:0019959

Also known as:glucagonoma syndromepancreatic glucagonoma

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Overview

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.

Clinical Trials2 registered

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Neoplasm of the pancreas; and common findings: Glossitis, Diabetes mellitus, Skin rash, and Pruritus and others. 42 total HPO annotations.

Organ System	Phenotype Count	Example Features
Digestive system	13	Diarrhea, Nausea and vomiting, Constipation

Diagnosis

Biomarker and diagnostic research for glucagonoma has been reported in the published literature.

Prognosis

Phenotype severity distribution: 1 very common feature, 18 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

2 clinical trials registered. Interventions under study include drug therapy and other interventions. Pipeline includes 1 PHASE2. Research is primarily sponsored by academic and government institutions.

35 publications have been identified in PubMed for glucagonoma. Kisho has analyzed 26 by research type. Research spans Case Report / Case Series (50%), Review / Meta-Analysis (38%), and Diagnostic / Biomarker (8%).

Research Type	Count	% of Total
Patient case studies	13

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:53 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:97280

GARD

Genetic and Rare Diseases Info Center

GARD:2496

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

glucagonoma

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

glucagonoma

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications