MCID: GLC036
MIFTS: 51

Glucagonoma malady

Gastrointestinal, Endocrine, Cancer categories

Summaries for Glucagonoma

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold... more...

MalaCards: Glucagonoma, also known as weight loss, necrolytic migratory erythema , diabetes, stomatitis, and diarrhea, is related to insulinoma and multiple endocrine neoplasia. An important gene associated with Glucagonoma is MADD (MAP-kinase activating death domain), and among its related pathways are Synthesis, Secretion, and Inactivation of Glucagon-like Peptide-1 (GLP-1) and Prostacyclin signalling through prostacyclin receptor. The compounds estrogen and secretoneurin have been mentioned in the context of this disorder. Affiliated tissues include skin, ovary and liver, and related mouse phenotypes are digestive/alimentary and reproductive system.

Aliases & Classifications for Glucagonoma

Sources:
43NIH Rare Diseases, 45Novoseek, 49Orphanet, 61UMLS, 26ICD10 via Orphanet, 36MESH via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Gastrointestinal, Endocrine


Aliases & Descriptions:

glucagonoma 43 45 49 61
weight loss, necrolytic migratory erythema , diabetes, stomatitis, and diarrhea 43
glucagonoma syndrome 43


External Ids:

ICD10 via Orphanet26 E16.8
MESH via Orphanet36 D005935
SNOMED-CT via Orphanet58 128855009, 16424000, 302823005
UMLS via Orphanet62 C0017689

Related Diseases for Glucagonoma

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Glucagonoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 163)
idRelated DiseaseScoreTop Affiliating Genes
1insulinoma30.6PCSK1, VIP, PCSK2, SST, SSTR2, SSTR5
2multiple endocrine neoplasia30.5CHGB, CHGA, MEN1, GAST, VIP
3gastrinoma30.2ENO2, SST, SSTR2, SSTR5, CHGA, GH1
4adenoma30.2GCG, SCG5, SCT, GAST, MEN1, GHRL
5primary hyperparathyroidism30.1MEN1, CHGA, SCT, GAST
6vipoma30.1GH1, GHRH, MEN1, GAST, SCT, VIP
7adrenal adenoma29.8MEN1
8apudoma29.8SST
9angiodysplasia29.8SST
10acinar cell carcinoma29.8GAST, CHGA
11hypercalcemia29.8SCT, GAST, MEN1, CHGA
12morbid obesity29.8GCG, GHRL, CCK
13pancreatitis, chronic29.8CCK, SCT, SST, GCG
14somatostatinoma29.8GAST, VIP, SST, SSTR2, CHGA, GHRH
15pancreatic islet cell tumors29.8PCSK1, SST, CHGA, MEN1, GAST, VIP
16prolactinoma29.8GH1, CHGB, SSTR5, SSTR2, SST, MEN1
17short bowel syndrome29.8GAST, CCK, GCG, GH1, SST
18anorexia nervosa29.8GH1, GCG, CCK, GHRL, GHRH
19hypoglycemia29.8SCT, GCG, GAST, GHRL, SST, CHGA
20pheochromocytoma29.8ENO2, VIP, SCG5, PCSK1, PCSK2, SST
21n syndrome10.6
22somatostatin analog10.3
23metastatic insulinoma10.2
24diabetic ketoacidosis10.1
25intestinal disease10.0SST
26phaeochromocytoma10.0CHGA
27duodenitis10.0GAST
28duodenum cancer10.0SST
29von hippel-lindau disease10.0MEN1
30esophagitis10.0GAST
31gastroduodenitis10.0GAST
32hirschsprung's disease10.0VIP
33ileus10.0GHRL
34addison's disease10.0PCSK1
35irritable bowel syndrome10.0CCK
36eating disorder10.0GHRL
37carney complex10.0MEN1
38cerebral primitive neuroectodermal tumor10.0ENO2
39subependymal giant cell astrocytoma10.0ENO2
40hemangioma10.0MEN1
41teratoma10.0ENO2
42thyroid cancer10.0CHGA, SST
43postcholecystectomy syndrome10.0GAST
44fibrous dysplasia10.0GH1
45cholelithiasis10.0SST, CCK
46pancreatic cholera10.0SST, VIP
47acth-secreting pituitary adenoma10.0GH1
48ganglioneuroblastoma10.0VIP, ENO2
49neuroblastoma10.0ENO2, VIP
50panic disorder10.0GHRH, CCK

Graphical network of the top 20 diseases related to Glucagonoma:



Diseases related to glucagonoma

Clinical Features for Glucagonoma

Drugs & Therapeutics for Glucagonoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

Search CenterWatch for Glucagonoma

Drug clinical trials:

Search ClinicalTrials for Glucagonoma

Search NIH Clinical Center for Glucagonoma

Search CenterWatch for Glucagonoma

Genetic Tests for Glucagonoma

Anatomical Context for Glucagonoma

Sources:
33MalaCards
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MalaCards organs/tissues related to Glucagonoma:

33
Skin, Ovary, Liver, Lymph node, Heart, Skeletal muscle, Pancreas, B cells, Pancreatic islet, Pituitary

Animal Models for Glucagonoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Glucagonoma

Sources:
51PubMed
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Articles related to Glucagonoma:

(show top 50)    (show all 279)
idTitleAuthorsYear
1
Putting the pieces together: necrolytic migratory erythema and the glucagonoma syndrome. (23681843)
2013
2
Necrolytic migratory erythema and glucagonoma rising from pancreatic head. (23890147)
2013
3
Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? (23259638)
2012
4
Glucagon cell adenomatosis: a new entity associated with necrolytic migratory erythema and glucagonoma syndrome. (21763589)
2011
5
Superficial necrolytic dermatitis associated with extrapancreatic glucagonoma in a dog. (19152590)
2009
6
Necrolytic migratory erythaema and glucagonoma syndrome. (19317246)
2009
7
What is your diagnosis? Necrolytic migratory erythema associated with a glucagonoma. (18306843)
2008
8
Necrolytic migratory erythema associated with glucagonoma: a report of 2 cases. (18438582)
2008
9
Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: report of a case. (19039643)
2008
10
Glucagonoma and pseudoglucagonoma syndrome. (15254346)
2004
11
Peripheral amino acid and fatty acid infusion for the treatment of necrolytic migratory erythema in the glucagonoma syndrome. (12460334)
2002
12
Amino acid, glucose, and lipid kinetics after palliative resection in a patient with glucagonoma syndrome. (11398151)
2001
13
Current imaging and possible therapeutic management of glucagonoma tumors: a case report. (10656647)
2000
14
Pancreatic glucagonoma: detection by positron emission tomography. (10724498)
2000
15
Protein metabolism in glucagonoma. (10096785)
1999
16
Asymptomatic glucagonoma presenting with an isolated hepatic nodule. (9756012)
1998
17
F-18 fluorodeoxyglucose positron emission tomography imaging in ectopic nonfunctioning glucagonoma. (9676959)
1998
18
Glucagonoma and pseudoglucagonoma syndromes. (9109001)
1997
19
Necrolytic migratory erythema without glucagonoma in patients with liver disease. (8647984)
1996
20
Glucagonoma associated with calculous pancreatitis. (8871247)
1996
21
Calcium reverses octreotide inhibition of insulin and glucagon levels in patients with insulinoma and glucagonoma. (8813473)
1996
22
Early detection of pancreatic glucagonoma. (8540521)
1995
23
An immunohistochemical study of glucagonoma conducted on the metastatic lymph nodes from a patient with recurrent metastatic glucagonoma: report of a case. (7894192)
1994
24
Glucagonoma syndrome with increased lactate dehydrogenase isoenzymes: octreotide treatment. (7528001)
1994
25
Acetylcholine regulates glucagon secretion from human glucagonoma cells. (7874280)
1994
26
Necrolytic migratory erythema without glucagonoma in a heroin-dependent patient. (8003793)
1994
27
Cystic pancreatic glucagonoma in contact with insulinoma found in a hypoglycemic patient. (1437839)
1992
28
A glucagonoma without necrolytic migratory erythema. (1594565)
1992
29
A malignant tumor of the pancreas producing glucagonoma syndrome: coexistence of glucagon and pancreatic polypeptide (PP) in the tumor cells. (2548179)
1989
30
A combined glucagonoma and VIPoma syndrome. First pathologic and clinical report. (2847862)
1988
31
Effect of somatostatin on skin lesions and concentrations of plasma amino acids in a patient with glucagonoma-syndrome. (2896149)
1988
32
Migratory erythema and glucagonoma: descriptive nomenclature. (2835921)
1988
33
Glucagonoma presenting as morbid obesity. (2848156)
1988
34
Cholecystokinin and glucagonoma. (2827024)
1988
35
Glucagonoma syndrome. Rapid response following arterial embolization of glucagonoma metastatic to the liver. (3030099)
1987
36
Use of a somatostatin analog (SMS 201-995) in the glucagonoma syndrome. (2878500)
1986
37
Necrolytic migratory erythema (glucagonoma syndrome). (6096544)
1984
38
Cytoreduction surgery for metastatic glucagonoma. A clinicopathologic study with special reference to the utility of immunocytochemistry and ultrastructure in diagnosis. (6092886)
1984
39
Glucagonoma occurring as a large cystic abdominal mass. (6326336)
1984
40
Report of 2 cases of glucagonoma syndrome with a brief review of literature. (6327197)
1983
41
A unique apudoma producing the glucagonoma and gastrinoma syndromes. (6878103)
1983
42
Refractory anaemia in the glucagonoma syndrome. (6276075)
1981
43
Glucagonoma tumors and syndrome. (6276079)
1981
44
A case with glucagonoma syndrome--endocrine and metabolic studies. (6273136)
1981
45
The glucagonoma syndrome. Report of a case. (6243459)
1980
46
Necrolytic migratory erythema, presenting as candidiasis, due to a pancreatic glucagonoma. (476569)
1979
47
Amino acid deficiency and the skin rash associated with glucagonoma. (111595)
1979
48
The glucagonoma syndrome. A distinctive cutaneous marker of systemic disease. (204256)
1978
49
Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome. (194537)
1977
50
Glucagonoma syndrome. Report of two cases and literature review. (194536)
1977

Genetic Variations for Glucagonoma

Expression for genes affiliated with Glucagonoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Glucagonoma

Search GEO for disease gene expression data for Glucagonoma.

Pathways for genes affiliated with Glucagonoma

Sources:
54Reactome, 12EMD Millipore, 38NCBI BioSystems Database, 30KEGG
See all sources

Compounds for genes affiliated with Glucagonoma

Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 24HMDB, 50PharmGKB, 60Tocris Bioscience
See all sources

Compounds related to Glucagonoma according to GeneCards/GeneDecks:

(show top 50)    (show all 217)
idCompoundScoreTop Affiliating Genes
1estrogen4510.3GCG, PCSK1
2secretoneurin459.9CHGB, CHGA, SCT, VIP
3111in-pentetreotide459.8SST, SSTR2, SSTR5, CHGA
4vapreotide29 45 1111.8SSTR5, SSTR2, CCK, SST
5minigastrin459.8VIP, SST, CCK, GAST
6lanreotide45 2910.8GHRH, GH1, CHGA, SSTR5, SST, SSTR2
7peptidylglycine459.7GAST, CHGA, CHGB, PCSK1, PCSK2
8norepinephrine45 11 2411.6CHGB, CHGA, GHRH, GAST, VIP, CCK
9acipimox45 2910.6GHRH, SST, GH1, GHRL, GCG
105-hydroxyindoleacetic acid45 2410.5SST, GAST, CHGA, ENO2, VIP
11loxiglumide459.5SST, GAST, CCK, SCT
12i-app459.5PDX1, GCG, PCSK1, PCSK2, GAST
13dtpa459.5GCG, SSTR2, GAST, SST, CCK
14naloxone45 50 29 1112.5GH1, VIP, CCK, GHRL, GHRH, SST
15omeprazole45 29 50 11 2413.4CHGA, SCT, SST, CCK, GAST
16proglucagon459.4CHGA, PCSK2, PCSK1, PDX1, GCG, SCG5
17propranolol45 29 50 11 2413.4GH1, VIP, SCT, GAST, SST, CHGA
18forskolin45 50 1111.3SSTR2, GHRH, SCT, PDX1, CCK, GCG
19thyroxine45 2410.2GH1, GHRH, SST, GAST, VIP, SCT
20atropine45 29 1111.2GHRH, GH1, GHRL, CCK, SCT, GAST
21amide459.2GHRH, CHGA, CCK, SCG5, GAST, GHRL
22pirenzepine45 29 1111.2GCG, CCK, GAST, GHRL, GHRH, GH1
23isoproterenol45 1110.0GCG, SST, CHGA, VIP, SCT, GAST
24carbachol45 29 1111.0VIP, GCG, SCT, GAST, GHRL, CHGA
25nifedipine45 50 29 1112.0VIP, GAST, GHRL, GHRH, CHGA, SST
26epinephrine45 11 2410.9GHRL, VIP, GCG, SCT, CHGA, GH1
27ribonucleic acid458.9GH1, SSTR5, GHRH, SSTR2, GHRL, CHGA
285-hydroxytryptamine458.9CCK, GAST, GHRL, CHGA, SST, ENO2
29gnrh458.8VIP, GCG, GAST, GHRL, GHRH, GH1
30gaba458.7GHRL, SCT, CCK, CHGA, SST, ENO2
31ibmx45 29 6010.7PCSK1, PCSK2, GH1, SCT, GCG, CCK
32dexamethasone45 50 29 1111.7VIP, ENO2, CCK, SCG5, GHRL, GHRH
33oxyntomodulin45 609.6GAST, GHRL, CHGA, SST, PCSK2, PCSK1
34histamine45 29 2410.6VIP, SCG5, SST, GCG, CCK, SCT
35guanine45 11 2410.5GCG, VIP, CHGA, SCT, SSTR2, GH1
36acetylcholine45 50 29 11 2412.5ENO2, GAST, SCT, GCG, VIP, CCK
37thymidine45 249.4GHRL, VIP, PDX1, SCG5, SCT, GAST
38c-peptide458.4PCSK1, PCSK2, SST, GCG, CCK, PDX1
39glucose458.3PCSK1, PCSK2, SSTR2, GHRH, GHRL, GAST
40testosterone45 60 11 2411.3GHRL, SCT, GAST, SCG5, GHRH, GH1
41dopamine45 29 11 2411.3CHGA, GHRH, GHRL, GAST, SCT, CCK
42vegf458.2GCG, GH1, PDX1, GAST, MEN1, GHRL
43glutamate458.2GHRL, CHGA, CCK, SCG5, SCT, GCG
44arginine458.1PCSK1, GHRL, VIP, GCG, CCK, SCG5
45octreotide45 60 29 1111.0VIP, GCG, CCK, SCT, GAST, GHRL
46adenylate457.9GHRL, GHRH, GH1, CHGA, SSTR2, SST
47tyrosine457.7MEN1, GHRL, CHGA, GAST, SCT, CHGB
48serine457.7PCSK1, SCG5, SCT, VIP, GCG, GAST
49acth457.6VIP, CCK, SCG5, SCT, GAST, GHRL
50calcium45 50 11 2410.6GHRH, GAST, VIP, CCK, PDX1, ENO2

GO Terms for genes affiliated with Glucagonoma

Sources:
16Gene Ontology
See all sources

Cellular components related to Glucagonoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.9SCG5, CHGA, CHGB
2secretory granule lumenGO:0347749.4PCSK1, PCSK2, GHRL, GCG
3perikaryonGO:0432049.4ENO2, PCSK2, CCK
4extracellular spaceGO:0056158.5GCG, CCK, GHRL, PCSK1, PCSK2, SST
5extracellular regionGO:0055767.8SST, VIP, GCG, CCK, SCG5, SCT

Biological processes related to Glucagonoma according to GeneCards/GeneDecks:

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of growth hormone secretionGO:06012410.2GHRL, GHRH
2growth hormone secretionGO:03025210.1GHRL, GHRH
3positive regulation of multicellular organism growthGO:04001810.0GHRL, GHRH, GH1
4glucose metabolic processGO:0060069.7ENO2, GHRL, PDX1
5negative regulation of cell proliferationGO:0082859.7SST, SSTR2, SSTR5, MEN1, PDX1
6cell-cell signalingGO:0072679.7PCSK1, SST, SSTR2, GHRH
7positive regulation of insulin-like growth factor receptor signaling pathwayGO:0435689.7GH1, GHRH
8regulation of insulin secretionGO:0507969.7GCG, SSTR5, PCSK1
9G-protein coupled receptor signaling pathwayGO:0071869.3SST, SSTR5, GHRL, GAST, GCG, VIP

Molecular functions related to Glucagonoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1somatostatin receptor activityGO:00499410.1SSTR5, SSTR2
2growth hormone-releasing hormone activityGO:0166089.8GHRH, GHRL
3hormone activityGO:0051798.5SCT, SST, CHGB, GH1, GAST, VIP

Products for genes affiliated with Glucagonoma

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Sources for Glucagonoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet