MCID: GLC036
MIFTS: 47

Glucagonoma malady

Rare diseases, Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories
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Summaries for Glucagonoma

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Wikipedia:65 A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold... more...

MalaCards based summary: Glucagonoma, also known as glucagonoma syndrome, is related to pancreatitis and angiodysplasia. An important gene associated with Glucagonoma is MADD (MAP-kinase activating death domain), and among its related pathways are G alpha (s) signalling events and Signaling by GPCR. The compounds naltrexone and secretoneurin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and skin, and related mouse phenotypes are homeostasis/metabolism and nervous system.

Aliases & Classifications for Glucagonoma

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Sources:
42NIH Rare Diseases, 44Novoseek, 48Orphanet, 62UMLS, 63UMLS via Orphanet, 35MESH via Orphanet, 26ICD10 via Orphanet
See all sources

Glucagonoma, Aliases & Descriptions:

Name: Glucagonoma 42 44 48 62
Glucagonoma Syndrome 42 62
 
Weight Loss, Necrolytic Migratory Erythema , Diabetes, Stomatitis, and Diarrhea 42


Classifications:



External Ids:

UMLS via Orphanet63 C0017689
MESH via Orphanet35 D005935
ICD10 via Orphanet26 E16.8

Related Diseases for Glucagonoma

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

(show top 50)    (show all 81)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis30.9SST, SCT
2angiodysplasia30.3SST
3insulinoma30.2SST, SCT, GHRH, SSTR5
4gastrinoma30.1SST, SCT, SSTR5
5somatostatinoma30.0SST, GHRH
6peptic ulcer disease29.9SCT, SST
7vipoma29.9SCT, SST, GHRH
8hypoglycemia29.6GHRH, SCT, SST
9prolactinoma29.6SSTR5, SST, CHGB
10pheochromocytoma29.3SST, SSTR5, CHGB, SCT
11adenoma29.2GHRH, SSTR5, CHGB, SCT, SST
12multiple endocrine neoplasia10.4
13hepatitis10.4
14dermatitis10.3
15gigantism10.3SST
16dumping syndrome10.3SST
17diabetes mellitus10.1
18islet cell tumor10.1
19liver disease10.1
20diabetic ketoacidosis10.1
21duodenitis10.1
22primary hyperparathyroidism10.1
23hyperparathyroidism10.1
24metastatic insulinoma10.1
25somatostatin analog10.1
26hyperinsulinemic hypoglycemia10.1SCT, SST
27steatorrhea10.0SST, SCT
28atrophic gastritis10.0SST, SCT
29serotonin syndrome10.0SST, GHRH
30duodenal ulcer10.0SST, SCT
31adrenal gland hyperfunction10.0SST, GHRH
32liver cirrhosis10.0GHRH, SCT
33hyperprolactinemia10.0SST, GHRH
34neuroendocrine tumor10.0GHRH, SST
35hypopituitarism10.0GHRH, SST
36cushing's syndrome10.0SST, GHRH
37acute pancreatitis10.0SCT, SST
38hyperthyroidism10.0GHRH, SST
39gastritis9.9SST, SCT
40paraganglioma9.9SST, CHGB
41dilated cardiomyopathy9.9
42hepatitis b9.9
43morbid obesity9.9
44myelodysplastic syndromes9.9
45obesity9.9
46acrodermatitis9.9
47adrenal cortical adenoma9.9
48heroin dependence9.9
49hypercalcemia9.9
50acrodermatitis enteropathica9.9

Graphical network of the top 20 diseases related to Glucagonoma:



Diseases related to glucagonoma

Symptoms for Glucagonoma

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Drugs & Therapeutics for Glucagonoma

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Drug clinical trials:

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Search NIH Clinical Center for Glucagonoma

Genetic Tests for Glucagonoma

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Anatomical Context for Glucagonoma

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MalaCards organs/tissues related to Glucagonoma:

32
Pancreas, Liver, Skin, Lymph node, Pancreatic islet, Bone, Heart, Ovary, Pituitary

Animal Models for Glucagonoma or affiliated genes

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MGI Mouse Phenotypes related to Glucagonoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053767.5CHGB, SSTR5, SST, GHRH, SCT
2MP:00036317.3MADD, GHRH, SST, CHGB, SCT

Publications for Glucagonoma

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Articles related to Glucagonoma:

(show top 50)    (show all 279)
idTitleAuthorsYear
1
Glucagonoma syndrome: a case report with focus on skin disorders. (25152626)
2014
2
Novel mutation 928G>C of MEN1 gene in a familial multiple endocrine neoplasia type 1 case (MEN1) with co-existence of insulinoma and glucagonoma. (25047095)
2014
3
Pancreatic glucagonoma metastasising to the right ovary five years after initial surgery: a case report. (24018597)
2013
4
Pancreatic glucagonoma with pancreatic calcification. (23719610)
2013
5
Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? (23259638)
2012
6
Bone lesions in recurrent glucagonoma: A case report and review of literature. (22737277)
2012
7
Necrolytic migratory erythema as the first manifestation of glucagonoma. (22970401)
2012
8
An unusual case of subclinical diffuse glucagonoma coexisting with two nodules in the pancreas: characteristic features on computed tomography. (22239827)
2012
9
Glucagonoma syndrome: a case report. (21859461)
2011
10
Necrolytic migratory erythema associated with glucagonoma syndrome. (20530273)
2010
11
Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). (19525592)
2009
12
Primary malignant hepatic glucagonoma: an autopsy case. (19367016)
2009
13
What is your diagnosis? Necrolytic migratory erythema associated with a glucagonoma. (18306843)
2008
14
Metastatic glucagonoma: treatment with liver transplantation. (16443073)
2006
15
Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. (16923138)
2006
16
Metastatic insulinoma and glucagonoma from the pancreas responsible for specific peritumoral patterns of hepatic steatosis secondary to local effects of insulin and glucagon on hepatocytes. (16247938)
2005
17
Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus. (12918465)
2003
18
Pancreatic glucagonoma as a rare cause of chronic obstructive pancreatitis. (11023587)
2000
19
Protein metabolism in glucagonoma. (10096785)
1999
20
Cystic glucagonoma with loss of heterozygosity on chromosome 11 in multiple endocrine neoplasia type 1. (9196616)
1997
21
Glucagonoma and pseudoglucagonoma syndromes. (9109001)
1997
22
Glucagonoma: case report and literature review]. (9201330)
1996
23
Rare presentation of endocrine pancreatic tumour: a case of glucagonoma without necrolytic migratory erythema? (8541575)
1995
24
The long-acting somatostatin analogue octreotide alleviates symptoms by reducing posttranslational conversion of prepro-glucagon to glucagon in a patient with malignant glucagonoma, but does not prevent tumor growth. (8186658)
1994
25
An immunohistochemical study of glucagonoma conducted on the metastatic lymph nodes from a patient with recurrent metastatic glucagonoma: report of a case. (7894192)
1994
26
Cystic pancreatic glucagonoma in contact with insulinoma found in a hypoglycemic patient. (1437839)
1992
27
Glucagonoma syndrome. (1935216)
1991
28
Necrolytic migratory erythema without glucagonoma: the role of dietary essential fatty acids. (1751353)
1991
29
Protein HMG-17 is hyper-expressed in rat glucagonoma. Single-step isolation and sequencing. (2169420)
1990
30
Electron microscopic and immunoelectron microscopic demonstration of pancreatic polypeptide cells in glucagonoma: colocalization of pancreatic peptide and glucagon in single secretory granules. (2537546)
1989
31
Octreotide (SMS 201-995) in the treatment of metastatic glucagonoma: report of one case and review of the literature. (2548911)
1989
32
Glucagonoma syndrome. Rapid response following arterial embolization of glucagonoma metastatic to the liver. (3030099)
1987
33
Immunocytochemical characterization of 10 pancreatic tumours, associated with the glucagonoma syndrome, using antibodies to separate regions of the pro-glucagon molecule and other neuroendocrine markers. (3007321)
1986
34
Use of a somatostatin analog (SMS 201-995) in the glucagonoma syndrome. (2878500)
1986
35
Secretory response and immunochemical heterogeneity of glucagon in plasma and tumor extracts of a patient with glucagonoma. (3005153)
1986
36
Carbohydrate antigen (CA 19.9) and malignant glucagonoma. (3024003)
1986
37
Disappearance of glucagonoma rash after surgical resection, but not during dietary normalization of serum amino acids. (6320631)
1984
38
Glucagonoma syndrome: case report and literature review. (6092443)
1984
39
Metastatic insulinoma with long survival and glucagonoma syndrome. (6318634)
1984
40
Successful treatment of metastatic glucagonoma with dacarbazine. (6142319)
1984
41
Glucagonoma and its angiographic diagnosis. (6301675)
1982
42
Use of somatostatin and somatostatin analogs in a patient with a glucagonoma. (6114963)
1981
43
Metastatic glucagonoma. improvement after surgical debulking. (6244750)
1980
44
Clinical and metabolic aspects of glucagonoma. (6987481)
1980
45
The glucagonoma syndrome: surgically curable diabetes. (215044)
1979
46
Necrolytic migratory erythema, presenting as candidiasis, due to a pancreatic glucagonoma. (476569)
1979
47
The glucagonoma syndrome and its management. (201809)
1977
48
The glucagonoma syndrome. (195450)
1977
49
Distribution of plasma glucagon immunoreactivity in a patient with suspected glucagonoma. (178682)
1976
50
Glucagonoma syndrome in a young man. (959215)
1976

Variations for Glucagonoma

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Expression for genes affiliated with Glucagonoma

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Expression patterns in normal tissues for genes affiliated with Glucagonoma

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Pathways for genes affiliated with Glucagonoma

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Compounds for genes affiliated with Glucagonoma

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Compounds related to Glucagonoma according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1naltrexone44 28 50 1112.8GHRH, SCT
2secretoneurin449.7SCT, CHGB
3dbc-amp449.7GHRH, SCT
4pasireotide44 1110.6SST, SSTR5
5(1r,1s,3r/1r,1r,3s)-l-054,264619.6SSTR5, SST
6cyclosomatostatin619.6SSTR5, SST
7cyn 154806619.6SSTR5, SST
8somatostatin61 2410.6SSTR5, SST
9vapreotide44 28 1111.6SSTR5, SST
10111in-pentetreotide449.6SSTR5, SST
11taurodeoxycholate449.6SST, SCT
12catecholamine449.6CHGB, SCT
13gold449.5GHRH, SCT
14lafutidine449.5SCT, SST
15vinblastine44 50 1111.5SST, MADD
16loxiglumide449.5SCT, SST
17ghrp449.5GHRH, SST
18hexarelin44 2810.5SST, GHRH
19pyridostigmine44 1110.5SST, GHRH
20pegvisomant44 1110.5GHRH, SST
21acipimox44 2810.5GHRH, SST
22pirenzepine44 28 1111.5SST, GHRH
23oxyntomodulin44 6110.5SST, SCT
24omeprazole44 50 28 24 1113.5SST, SCT
25bromocriptine44 28 1111.5SST, GHRH
26naloxone44 28 50 1112.5GHRH, SST
27c-peptide449.4SST, SCT
28levodopa44 1110.4SST, GHRH
29nifedipine44 28 50 1112.2GHRH, SST
30ibmx44 61 2811.1SST, SCT
31lanreotide44 2810.1SSTR5, SST, GHRH
32clonidine44 50 28 1112.0SCT, SST, GHRH
33atropine44 28 1111.0GHRH, SST, SCT
34propranolol44 28 50 24 1113.0SCT, SST, GHRH
35carbachol44 28 1111.0SCT, SST
36thyroxine44 2410.0SCT, SST, GHRH
37adenylate448.9GHRH, SST, SCT
38testosterone44 61 24 1111.9GHRH, SST, SCT
39isoproterenol44 119.8SST, SCT
40arginine448.8SCT, SST, GHRH
41glutamate448.8SCT, SST, GHRH
42dexamethasone44 50 28 1111.7GHRH, SST, CHGB
43glucose448.6GHRH, SST, SCT
44octreotide44 61 28 1111.6SCT, SSTR5, SST, GHRH
45calcium44 50 24 1111.5GHRH, SSTR5, CHGB, SCT
46norepinephrine44 24 1110.4GHRH, SST, CHGB, SCT
47acth448.0GHRH, SST, SSTR5, CHGB, SCT
48dopamine44 28 24 1111.0GHRH, SST, SSTR5, CHGB, SCT

GO Terms for genes affiliated with Glucagonoma

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Cellular components related to Glucagonoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.0GHRH, SST, CHGB, SCT

Biological processes related to Glucagonoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell surface receptor signaling pathwayGO:0071669.2MADD, SST

Molecular functions related to Glucagonoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:0051798.5SST, CHGB, SCT

Products for genes affiliated with Glucagonoma

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Sources for Glucagonoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet