Glucagonoma

Summary

Glucagonoma (MONDO:0019959) is a disease and 16 clinical trials. Top therapeutic interventions include edotreotide gallium ga-68, gefitinib, and octreotide acetate. A subtype of pancreatic neuroendocrine tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 42
• Clinical trials: 16

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.005	Europe	Validated
Point prevalence	<1 / 1 000 000		Europe	Validated

Signs & symptoms

Clinical features (HPO)

42 HPO clinical features (Orphanet curated; top 42 by frequency):

HPO ID	Term	Frequency
HP:0002894	Neoplasm of the pancreas	Very frequent (80-99%)
HP:0000206	Glossitis	Frequent (30-79%)
HP:0000819	Diabetes mellitus	Frequent (30-79%)
HP:0000988	Skin rash	Frequent (30-79%)
HP:0000989	Pruritus	Frequent (30-79%)
HP:0001824	Weight loss	Frequent (30-79%)
HP:0001895	Normochromic anemia	Frequent (30-79%)
HP:0001927	Acanthocytosis	Frequent (30-79%)
HP:0002014	Diarrhea	Frequent (30-79%)
HP:0002017	Nausea and vomiting	Frequent (30-79%)
HP:0002019	Constipation	Frequent (30-79%)
HP:0002039	Anorexia	Frequent (30-79%)
HP:0002240	Hepatomegaly	Frequent (30-79%)
HP:0002574	Episodic abdominal pain	Frequent (30-79%)
HP:0004396	Poor appetite	Frequent (30-79%)
HP:0008066	Abnormal blistering of the skin	Frequent (30-79%)
HP:0010280	Stomatitis	Frequent (30-79%)
HP:0012432	Chronic fatigue	Frequent (30-79%)
HP:0031181	Necrolytic migratory erythema	Frequent (30-79%)
HP:0000716	Depression	Occasional (5-29%)
HP:0001046	Intermittent jaundice	Occasional (5-29%)
HP:0001406	Intrahepatic cholestasis	Occasional (5-29%)
HP:0001438	Abnormality of abdomen morphology	Occasional (5-29%)
HP:0001541	Ascites	Occasional (5-29%)
HP:0001907	Thromboembolism	Occasional (5-29%)
HP:0002239	Gastrointestinal hemorrhage	Occasional (5-29%)
HP:0002570	Steatorrhea	Occasional (5-29%)
HP:0005214	Intestinal obstruction	Occasional (5-29%)
HP:0012334	Extrahepatic cholestasis	Occasional (5-29%)
HP:0030145	Lack of bowel sounds	Occasional (5-29%)
HP:0030895	Abnormal gastrointestinal motility	Occasional (5-29%)
HP:0000820	Abnormality of the thyroid gland	Very rare (<1-4%)
HP:0000837	Increased circulating gonadotropin level	Very rare (<1-4%)
HP:0000845	Elevated circulating growth hormone concentration	Very rare (<1-4%)
HP:0000870	Increased circulating prolactin concentration	Very rare (<1-4%)
HP:0001031	Subcutaneous lipoma	Very rare (<1-4%)
HP:0002893	Pituitary adenoma	Very rare (<1-4%)
HP:0002897	Parathyroid adenoma	Very rare (<1-4%)
HP:0003072	Hypercalcemia	Very rare (<1-4%)
HP:0003118	Increased circulating cortisol level	Very rare (<1-4%)
HP:0008200	Primary hyperparathyroidism	Very rare (<1-4%)
HP:0008256	Adrenocortical adenoma	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	glucagonoma
Mondo ID	MONDO:0019959
MeSH	D005935
Orphanet	97280
ICD-11	1918063179, 2121897507
NCIT	C95597
SNOMED CT	16424000
UMLS	C0017689
MedGen	4908
GARD	0002496
MedDRA	10018404
Is cancer (heuristic)	no

Also known as: glucagonoma · glucagonoma syndrome · pancreatic glucagonoma

Disease family

This is a subtype of pancreatic neuroendocrine tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › digestive system neuroendocrine neoplasm › digestive system neuroendocrine tumor, grade 1/2 › pancreatic neuroendocrine tumor › glucagonoma

Related subtypes (10): pancreatic delta cell neuroendocrine tumor, pancreatic gastrin-producing neuroendocrine tumor, non-functional pancreatic neuroendocrine tumor, pancreatic insulin-producing neuroendocrine tumor, somatostatinoma, GRFoma, PPoma, VIPoma, pancreatic neuroendocrine tumor G1, functional pancreatic neuroendocrine tumor

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 16.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	10
PHASE1	5
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01824875	PHASE2	ACTIVE_NOT_RECRUITING	Temozolomide With or Without Capecitabine in Treating Patients With Advanced Pancreatic Neuroendocrine Tumors
NCT00075439	PHASE2	COMPLETED	Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors
NCT00084461	PHASE2	TERMINATED	Romidepsin in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors
NCT00131911	PHASE2	COMPLETED	Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors
NCT00454363	PHASE2	COMPLETED	Pazopanib Hydrochloride in Treating Patients With Advanced Neuroendocrine Cancer
NCT01525082	PHASE2	COMPLETED	Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors
NCT02031536	PHASE2	TERMINATED	Everolimus in Patients With Pancreatic Neuroendocrine Tumors Metastatic to the Liver Previously Treated With Surgery
NCT02108782	PHASE2	WITHDRAWN	Dovitinib Lactate in Treating Patients With Pancreatic Neuroendocrine Tumors
NCT02259725	PHASE2	COMPLETED	Regorafenib in Treating Patients With Advanced or Metastatic Neuroendocrine Tumors
NCT02273752	PHASE2	TERMINATED	Pharmacokinetically Guided Everolimus in Patients With Breast Cancer, Pancreatic Neuroendocrine Tumors, or Kidney Cancer
NCT00004074	PHASE1	COMPLETED	Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu
NCT00655655	PHASE1	COMPLETED	Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors
NCT01204476	PHASE1	COMPLETED	Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma
NCT02831179	PHASE1	WITHDRAWN	Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor
NCT03147768	PHASE1	COMPLETED	Laser Tissue Welding - Distal Pancreatectomy Sealing Study
NCT03583528	Not specified	ACTIVE_NOT_RECRUITING	DOTATOC PET/CT for Imaging NET Patients

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
EDOTREOTIDE GALLIUM GA-68	4	1
GEFITINIB	4	1
OCTREOTIDE ACETATE	4	1
PAZOPANIB HYDROCHLORIDE	4	1
REGORAFENIB	4	1
ROMIDEPSIN	4	1
SORAFENIB	4	1
VATALANIB	3	2
VELIPARIB	3	2
CIXUTUMUMAB	2	1
DOVITINIB LACTATE	2	1
EDODEKIN ALFA	2	1
CHEMBL4066465	0	1