Details of Disease

Disease Name

Glucagonoma

glucagonoma syndrome; pancreatic glucagonoma; glucagonoma

Definition

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.

Disease Hierarchy

DISDMPU0: Pancreatic neuroendocrine tumor

DISDU90K: Glucagonoma

Disease Identifiers

MONDO ID

MONDO_0019959

MESH ID

D005935

UMLS CUI

C0017689

MedGen ID

4908

HPO ID

HP:0030404

Orphanet ID

97280

SNOMED CT ID

16424000

General Information of Disease (ID: DISDU90K)

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Romidepsin	DMT5GNL	Approved	Small molecular drug	[1]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 5 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
GCG	TT6Y4PN	Limited	Biomarker	[2]
GPR119	TT7QNVC	Limited	Altered Expression	[3]
FFAR1	TTB8FUC	Strong	Altered Expression	[4]
SSTR5	TT2BC4G	Strong	Altered Expression	[5]
GCGR	TT9O6WS	Definitive	Genetic Variation	[6]
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This Disease Is Related to 3 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
HMGN2	OTN20MEF	Limited	Altered Expression	[7]
MEN1	OTN6U6V0	Limited	Biomarker	[8]
PARP14	OTFXJAKK	Strong	Biomarker	[9]
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References

1	Romidepsin FDA Label
2	Spleen-preserving distal pancreatectomy and lymphadenectomy for glucagonoma syndrome: A case report.Medicine (Baltimore). 2019 Sep;98(38):e17037. doi: 10.1097/MD.0000000000017037.
3	GPR119 expression in normal human tissues and islet cell tumors: evidence for its islet-gastrointestinal distribution, expression in pancreatic beta and alpha cells, and involvement in islet function.Metabolism. 2013 Jan;62(1):70-8. doi: 10.1016/j.metabol.2012.06.010. Epub 2012 Aug 9.
4	Expression of the gene for a membrane-bound fatty acid receptor in the pancreas and islet cell tumours in humans: evidence for GPR40 expression in pancreatic beta cells and implications for insulin secretion.Diabetologia. 2006 May;49(5):962-8. doi: 10.1007/s00125-006-0193-8. Epub 2006 Mar 9.
5	Expression of somatostatin receptor types 1-5 in 81 cases of gastrointestinal and pancreatic endocrine tumors. A correlative immunohistochemical and reverse-transcriptase polymerase chain reaction analysis.Virchows Arch. 2002 May;440(5):461-75. doi: 10.1007/s00428-002-0609-x. Epub 2002 Mar 23.
6	Glucagon and Amino Acids Are Linked in a Mutual Feedback Cycle: The Liver--Cell Axis.Diabetes. 2017 Feb;66(2):235-240. doi: 10.2337/db16-0994.
7	Protein HMG-17 is hyper-expressed in rat glucagonoma. Single-step isolation and sequencing.Eur J Biochem. 1990 Aug 28;192(1):81-6. doi: 10.1111/j.1432-1033.1990.tb19198.x.
8	Foxa2, a novel protein partner of the tumour suppressor menin, is deregulated in mouse and human MEN1 glucagonomas.J Pathol. 2017 May;242(1):90-101. doi: 10.1002/path.4885. Epub 2017 Mar 27.
9	PARP-14 Promotes Survival of Mammalian but Not Pancreatic Cells Following Cytokine Treatment.Front Endocrinol (Lausanne). 2019 May 3;10:271. doi: 10.3389/fendo.2019.00271. eCollection 2019.