MCID: GLC036
MIFTS: 40

Glucagonoma malady

Rare diseases, Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories

Summaries for Glucagonoma

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Wikipedia:63 A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in the overproduction of... more...

MalaCards based summary: Glucagonoma, also known as weight loss, necrolytic migratory erythema , diabetes, stomatitis, and diarrhea, 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:
41NIH Rare Diseases, 43Novoseek, 47Orphanet, 60UMLS, 61UMLS via Orphanet, 34MESH via Orphanet, 26ICD10 via Orphanet
See all sources

Glucagonoma, Aliases & Descriptions:

Name: Glucagonoma 41 43 47 60
Weight Loss, Necrolytic Migratory Erythema , Diabetes, Stomatitis, and Diarrhea 41
 
Glucagonoma Syndrome 41


Classifications:



Characteristics (Orphanet epidemiological data):

47
glucagonoma:
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adult; Age of death: adult


External Ids:

Orphanet47 97280
UMLS via Orphanet61 C0017689
MESH via Orphanet34 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 Suite gene sharing:

(show top 50)    (show all 81)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis30.9SST, SCT
2angiodysplasia30.3SST
3insulinoma30.2SCT, SSTR5, SST, GHRH
4gastrinoma30.1SST, SSTR5, SCT
5somatostatinoma30.0GHRH, SST
6peptic ulcer disease29.9SCT, SST
7vipoma29.9SCT, SST, GHRH
8hypoglycemia29.6SCT, SST, GHRH
9prolactinoma29.6SST, SSTR5, CHGB
10pheochromocytoma29.3SCT, CHGB, SSTR5, SST
11adenoma29.2SCT, CHGB, SSTR5, SST, GHRH
12multiple endocrine neoplasia10.4
13hepatitis10.4
14dermatitis10.3
15dumping syndrome10.3SST
16gigantism10.3SST
17dilated cardiomyopathy10.1
18islet cell tumor10.1
19liver disease10.1
20diabetic ketoacidosis10.1
21duodenitis10.1
22primary hyperparathyroidism10.1
23hyperparathyroidism10.1
24metastatic insulinoma10.1
25alcohol dependence10.1GHRH, SCT
26hyperinsulinemic hypoglycemia, familial, 210.0SST, SCT
27steatorrhea10.0SST, SCT
28atrophic gastritis10.0SCT, SST
29serotonin syndrome10.0GHRH, SST
30duodenal ulcer10.0SCT, SST
31adrenal gland hyperfunction10.0SST, GHRH
32liver cirrhosis10.0GHRH, SCT
33neuroendocrine tumor10.0SST, GHRH
34hyperprolactinemia10.0SST, GHRH
35hypopituitarism10.0SST, GHRH
36cushing's syndrome10.0GHRH, SST
37acute pancreatitis10.0SST, SCT
38hyperthyroidism10.0GHRH, SST
39gastritis10.0SST, SCT
40muscle hypertrophy9.9
41myelodysplastic syndrome9.9
42obesity9.9
43acrodermatitis enteropathica9.9
44anorexia nervosa9.9
45hepatitis b9.9
46morbid obesity9.9
47acrodermatitis9.9
48adrenal cortical adenoma9.9
49appendiceal l-cell glucagon-like peptide producing tumor9.9
50short bowel syndrome9.9

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:

Search ClinicalTrials for Glucagonoma

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:

31
Pancreas, Liver, Skin, Lymph node, Heart, Pancreatic islet, Bone, Ovary, Pituitary

Animal Models for Glucagonoma or affiliated genes

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MGI Mouse Phenotypes related to Glucagonoma:

35
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 284)
idTitleAuthorsYear
1
Glucagonoma syndrome: a case report with focus on skin disorders. (25152626)
2014
2
Novel mutation 928G&gt;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
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
16
Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus. (12918465)
2003
17
Glucagonoma Syndrome (25905270)
2000
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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Search GEO for disease gene expression data for Glucagonoma.

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 Suite gene sharing:

(show all 48)
idCompoundScoreTop Affiliating Genes
1naltrexone43 28 49 1212.8GHRH, SCT
2secretoneurin439.7SCT, CHGB
3dbc-amp439.7GHRH, SCT
4pasireotide43 1210.6SST, SSTR5
5(1r,1s,3r/1r,1r,3s)-l-054,264599.6SSTR5, SST
6cyclosomatostatin599.6SSTR5, SST
7cyn 154806599.6SSTR5, SST
8somatostatin59 2410.6SSTR5, SST
9vapreotide43 28 1211.6SSTR5, SST
10111in-pentetreotide439.6SSTR5, SST
11taurodeoxycholate439.6SST, SCT
12catecholamine439.6CHGB, SCT
13gold439.5GHRH, SCT
14lafutidine439.5SCT, SST
15vinblastine43 49 1211.5SST, MADD
16loxiglumide439.5SCT, SST
17ghrp439.5GHRH, SST
18hexarelin43 2810.5SST, GHRH
19pyridostigmine43 1210.5SST, GHRH
20pegvisomant43 1210.5GHRH, SST
21acipimox43 2810.5GHRH, SST
22pirenzepine43 28 1211.5SST, GHRH
23oxyntomodulin43 5910.5SST, SCT
24omeprazole43 49 28 24 1213.5SST, SCT
25bromocriptine43 28 1211.5SST, GHRH
26naloxone43 28 49 1212.5GHRH, SST
27c-peptide439.4SST, SCT
28levodopa43 1210.4SST, GHRH
29nifedipine43 28 49 1212.2GHRH, SST
30ibmx43 59 2811.1SST, SCT
31lanreotide43 2810.1SSTR5, SST, GHRH
32clonidine43 49 28 1212.0SCT, SST, GHRH
33atropine43 28 1211.0GHRH, SST, SCT
34propranolol43 28 49 24 1213.0SCT, SST, GHRH
35carbachol43 28 1211.0SCT, SST
36thyroxine43 2410.0SCT, SST, GHRH
37adenylate438.9GHRH, SST, SCT
38testosterone43 59 24 1211.9GHRH, SST, SCT
39isoproterenol43 129.8SST, SCT
40arginine438.8SCT, SST, GHRH
41glutamate438.8SCT, SST, GHRH
42dexamethasone43 49 28 1211.7GHRH, SST, CHGB
43glucose438.6GHRH, SST, SCT
44octreotide43 59 28 1211.6SCT, SSTR5, SST, GHRH
45calcium43 49 24 1211.5GHRH, SSTR5, CHGB, SCT
46norepinephrine43 24 1210.4GHRH, SST, CHGB, SCT
47acth438.0GHRH, SST, SSTR5, CHGB, SCT
48dopamine43 28 24 1211.0GHRH, SST, SSTR5, CHGB, SCT

GO Terms for genes affiliated with Glucagonoma

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Cellular components related to Glucagonoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00055768.0GHRH, SST, CHGB, SCT

Biological processes related to Glucagonoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cell surface receptor signaling pathwayGO:00071669.2MADD, SST

Molecular functions related to Glucagonoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:00051798.5SST, 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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet