MCID: INS001
MIFTS: 64

Insulinoma malady

Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories

Summaries for Insulinoma

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8Disease Ontology, 63Wikipedia, 32MalaCards
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Wikipedia:63 An insulinoma is a tumor of the pancreas that is derived from beta cells and secretes insulin. more...

MalaCards: Insulinoma, also known as islet cell adenoma, is related to pancreatitis and hypoglycemia. An important gene associated with Insulinoma is ITS (Insulinoma tumor suppressor gene locus), and among its related pathways are Signaling by GPCR and Class A/1 (Rhodopsin-like receptors). The compounds glucose and calcium have been mentioned in the context of this disorder. Affiliated tissues include pancreas, testes and pancreatic islet, and related mouse phenotypes are homeostasis/metabolism and growth/size.

Disease Ontology:8 An adenoma that is a tumor in the pancreas that produces too much insulin.

Aliases & Classifications for Insulinoma

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8Disease Ontology, 42NIH Rare Diseases, 46OMIM, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 56SNOMED-CT, 39NCIt, 34MeSH, 26ICD10 via Orphanet, 35MESH via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

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


Aliases & Descriptions:

insulinoma 8 42 46 10 44 48 60
islet cell adenoma 8 60
insulin-producing tumor of islet cells 8
adenoma islet cell 44
insulinoma nos 8
insuloma 48


External Ids:

Disease Ontology8 DOID:3892
NCIt39 C3140
MeSH34 D007340
ICD10 via Orphanet26 E16.8
MESH via Orphanet35 D007340
SNOMED-CT via Orphanet57 25324008, 302822000
UMLS via Orphanet61 C0021670

Related Diseases for Insulinoma

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

(show top 50)    (show all 107)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis31.1CASR, SCT, SST
2hypoglycemia30.9GLP1R, GCK, IAPP, SCT, SST
3diabetes mellitus30.5PTPRN, GCK, CASR, PAX4, INSM2, GLP1R
4gastrinoma30.5GLP1R, SST, IAPP, MEN1, SCT, CASR
5type 2 diabetes mellitus30.4PTPRN, PAX4, SCT, IAPP, GLP1R, GCK
6hyperinsulinism30.3IAPP, GCK, SCT, SST, GLP1R, PAX4
7obesity30.3GLP1R, GCK, IAPP
8hyperinsulinemic hypoglycemia30.3SCT, SST
9neuroendocrine tumor30.2MEN1, SST, GLP1R, SCT, INSM1
10pheochromocytoma30.2MEN1, SCT, IAPP, SST, SSTR4, SSTR5
11hyperparathyroidism30.1CASR, SCT, MEN1
12hypercalcemia30.0SCT, MEN1, IAPP, CASR
13zollinger-ellison syndrome30.0SST, MEN1, SCT
14primary hyperparathyroidism30.0SCT, MEN1, CASR
15pancreatic cancer30.0SSTR5, SCT, MEN1, IAPP, SST
16hyperglycemia29.8PTPRN, SST, IAPP, GCK, PAX4, SCT
17adenoma29.8GIPR, SSTR5, SSTR4, MEN1, CASR, SCT
18pituitary adenoma29.8SST, CASR, MEN1, SSTR5
19glucose intolerance29.8GLP1R, IAPP, GCK
20lung cancer29.8SST
21peptic ulcer29.8SST, SCT
22dumping syndrome29.8SST
23hepatitis10.4
24duodenitis10.3
25hepatitis a10.2
26islet cell tumor10.2
27syndromic diarrhea10.1
28gastroduodenitis10.1
29hypokalemia10.1
30diarrhea10.1
31sdha-related hereditary paraganglioma-pheochromocytoma syndrome10.1
32metastatic insulinoma10.1
33duodenal ulcer10.0
34adenocarcinoma10.0
35hypertension10.0
36neuropathy10.0
37adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia10.0
38type 1 diabetes10.0PTPRN, PAX4, IAPP, WFS1, SCT, GCK
39carcinoid syndrome10.0SCT, MEN1, IAPP, SSTR5, SST, GLP1R
40pituitary tumors10.0SSTR4, SST, SSTR5, MEN1, INSM1
41thyroid medullary carcinoma10.0CASR, MEN1, IAPP, SSTR5, SST
42insulin resistance10.0GCK, PAX4, IAPP, GLP1R, SST
43glucagonoma10.0SCT, MEN1, SSTR5, SST
44prolactinoma10.0SST, SSTR4, SSTR5, MEN1
45vipoma10.0SCT, SST, MEN1
46atrophic gastritis10.0MEN1, SCT, SST
47acromegaly10.0SST, MEN1, SSTR5
48lung small cell carcinoma10.0SCT, MEN1, INSM1, SST
49pancreatic islet cell tumors10.0MEN1, SST, IAPP
50maturity-onset diabetes of the young10.0PAX4, GCK

Graphical network of the top 20 diseases related to Insulinoma:



Diseases related to insulinoma

Clinical Features for Insulinoma

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Drugs & Therapeutics for Insulinoma

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Insulinoma

Drug clinical trials:

Search ClinicalTrials for Insulinoma

Search NIH Clinical Center for Insulinoma

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Genetic Tests for Insulinoma

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Anatomical Context for Insulinoma

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Sources:
30LifeMap Discovery™, 32MalaCards
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MalaCards organs/tissues related to Insulinoma:

32
Pancreas, Testes, Pancreatic islet, Liver, T cells, Pituitary, Spleen, Brain, B cells, Lung, Lymph node, Appendix, Hypothalamus, Bone, Retina, Temporal lobe

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Insulinoma:
id TissueAnatomical CompartmentCell Relevance
1 PancreasIslets of LangerhansMature Beta Cells Potential therapeutic candidate, affected by disease

Animal Models for Insulinoma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Insulinoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537611.3PTPRN, KCNA5, GCK, CASR, SCT, MEN1
2MP:000537811.0CASR, MEN1, WFS1, IAPP, SSTR5, SST
3MP:000537910.8GCK, CASR, MEN1, WFS1, SSTR5, MAFA

Publications for Insulinoma

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Sources:
50PubMed
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Articles related to Insulinoma:

(show top 50)    (show all 743)
idTitleAuthorsYear
1
Sporadic insulinoma in a 10-year-old boy: a case report and literature review. (24413786)
2014
2
Canine insulinoma as a model for studying molecular genetics of tumorigenesis and metastasis. (23707578)
2013
3
Cellular biophysical dynamics and ion channel activities detected by AFM-based nanorobotic manipulator in insulinoma I^-cells. (23178285)
2013
4
Insulinoma: only in adults?-case reports and literature review. (23604412)
2013
5
Prolonged survival in an aged Labrador retriever with a metastatic insulinoma. (23325593)
2013
6
Malignant insulinoma: recommendations for characterisation and treatment. (23993836)
2013
7
The role of proinsulin and insulin in the diagnosis of insulinoma: a critical evaluation of the endocrine society clinical practice guideline. (24081736)
2013
8
Effects of pre-operative administration of medetomidine on plasma insulin and glucose concentrations in healthy dogs and dogs with insulinoma. (23714015)
2013
9
Liver transplantation for hepatic metastatic pancreatic insulinoma with a survival over five years. (22931990)
2012
10
A new frameshift MEN1 gene mutation associated with familial malignant insulinomas. (21184284)
2011
11
Modeling pharmacological inhibition of mast cell degranulation as a therapy for insulinoma. (22131884)
2011
12
Setting the bar for laparoscopic resection of sporadic insulinoma. (21293961)
2011
13
Amelioration of hypoglycemia with octreotide therapy in metastatic insulinoma with positive octreotide scan. (21160385)
2011
14
Distribution patterns of calcium-binding proteins in pancreatic tissue of non-diabetic as well as type 2 diabetic rats and in rat insulinoma beta-cells (INS-1). (20607274)
2010
15
Diagnosis and localisation of insulinoma: the value of modern magnetic resonance imaging in conjunction with calcium stimulation catheterisation. (20207727)
2010
16
Insulinoma in a patient with MEN 1 syndrome--9-year follow-up: case report. (20464709)
2010
17
Autoantibodies to insulin, insulinoma-associated antigen-2, and zinc transporter 8 improve the prediction of early insulin requirement in adult-onset autoimmune diabetes. (20061424)
2010
18
Localization of insulinoma associated protein 2, IA-2 in mouse neuroendocrine tissues using two novel monoclonal antibodies. (19233214)
2009
19
Insulinoma: a rare neuroendocrine pancreatic tumor. (20187464)
2009
20
Malignant insulinoma arising from intrasplenic heterotopic pancreas. (19454827)
2009
21
What should be done in an operating room when an insulinoma cannot be found? (19170712)
2009
22
Chronic reduction of the cytosolic or mitochondrial NAD(P)-malic enzyme does not affect insulin secretion in a rat insulinoma cell line. (19858194)
2009
23
Insulinoma: an evaluation by a single institution. (19051468)
2008
24
An unusual case of concurrent insulinoma and nesidioblastosis. (18762698)
2008
25
Glucose-stimulated insulin secretion of insulinoma INS-1E cells is associated with elevation of both respiration and mitochondrial membrane potential. (18248766)
2008
26
Re: "Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome". (17171485)
2007
27
Insulinoma in a dog with pre-existing insulin-dependent diabetes mellitus. (17209088)
2007
28
Laparoscopic versus open approach for solitary insulinoma. (17008952)
2007
29
Hypercalcitoninemia in a patient with a recurrent goitre and insulinoma: a case report. (16804794)
2006
30
Malignant insulinoma. (16431296)
2006
31
Well-differentiated pancreatic tumor/carcinoma: insulinoma. (17312378)
2006
32
EUS-guided alcohol ablation of an insulinoma. (16733126)
2006
33
Telerobotic-assisted laparoscopic spleen-preserving partial resection of the pancreatic tail for insulinoma. (18333283)
2006
34
Chromosomal instability predicts metastatic disease in patients with insulinomas. (15947114)
2005
35
Insulinoma, a rare neuroendocrine tumor: a case report. (15751752)
2005
36
Abnormalities of proinsulin processing in functioning insulinomas: clinical implications. (15473873)
2004
37
Frequent loss of heterozygosity at MEN-1 gene and chromosome 22q in insulinomas and its significance]. (15569430)
2004
38
Effect of prostaglandins against alloxan-induced cytotoxicity to insulin secreting insulinoma RIN cells in vitro. (15380818)
2004
39
Laparoscopic resection of insulinoma: two case reports. (15377443)
2004
40
Visualization of the specific interaction of sulfonylurea-incorporated polymer with insulinoma cell line MIN6. (15047719)
2004
41
An insulinoma for which secretin test and selective arterial calcium injection test were useful. (12413006)
2002
42
The effectiveness of administering a minimal dose of octreotide long-term prior to surgery for insulinoma: report of a case. (10883468)
2000
43
Insulinoma with hyperproinsulinemia during hypoglycemia and loss of expression of vacuolar-type H(+)-ATPase (V-ATPase) in the tumor tissue. (10211416)
1999
44
Immunolocalization of secretogranin II and insulin in a nerve growth factor-differentiated insulinoma cell line. (7641727)
1995
45
Localization of insulinomas to regions of the pancreas by intra-arterial stimulation with calcium. (7611592)
1995
46
Stimulatory effects of glucagon-like peptides on human insulinoma cells and insulin-releasing clonal RINm5F cells. (1965428)
1990
47
Identification of a human insulinoma cDNA encoding a novel mammalian protein structurally related to the yeast dibasic processing protease Kex2. (2154467)
1990
48
Amyloid fibrils in human insulinoma and islets of Langerhans of the diabetic cat are derived from a neuropeptide-like protein also present in normal islet cells. (3035556)
1987
49
A case of insulin autoimmune syndrome associated with small insulinomas and rheumatoid arthritis. (6266819)
1980
50
Control of refractory fasting hypoglycemia in a patient with suspected insulinoma with diphenylhydantoin. (4358644)
1974

Genetic Variations for Insulinoma

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Expression for genes affiliated with Insulinoma

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Insulinoma

Search GEO for disease gene expression data for Insulinoma.

Pathways for genes affiliated with Insulinoma

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Sources:
53Reactome, 29KEGG, 37NCBI BioSystems Database
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Compounds for genes affiliated with Insulinoma

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44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB, 59Tocris Bioscience, 28IUPHAR
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Compounds related to Insulinoma according to GeneCards/GeneDecks:

(show top 50)    (show all 53)
idCompoundScoreTop Affiliating Genes
1glucose4411.5PAX4, GLP1R, SST, IAPP, SCT, CASR
2calcium44 49 11 2414.3MEN1, WFS1, GLP1R, SCT, KCNA5, SSTR5
3c-peptide4411.3GLP1R, SST, IAPP, SCT, GCK, PTPRN
4adenylate4411.3SCT, GLP1R, CASR, SST, IAPP, GCK
5acth4411.2GIPR, SST, SCT, CASR, SSTR5
6potassium44 11 2413.2CASR, SCT, KCNA5, GLP1R, GCK
7octreotide44 59 28 1114.2SCT, SSTR5, SSTR4, SST
8arginine4411.1GCK, SST, SCT, PTPRN, CASR, IAPP
9cyn 1548065911.1SST, SSTR5, SSTR4
10cyclosomatostatin5911.1SSTR5, SST, SSTR4
11(1r,1s,3r/1r,1r,3s)-l-054,2645911.1SST, SSTR4, SSTR5
12somatostatin59 2412.1SSTR5, SSTR4, SST
13incretin4411.1GCK, GLP1R, IAPP
14oxyntomodulin44 5912.1SST, SCT, GLP1R
15lanreotide44 2812.1SSTR4, SST, SSTR5
16sulfonylurea4411.1GLP1R, IAPP, GCK
17[125i]tyr11-srif-142811.0SSTR4, SSTR5
18prl-29152811.0SSTR4, SSTR5
19des-aa5-[d-trp8]srif2811.0SSTR5, SSTR4
20srif-282811.0SSTR5, SSTR4
21[125i]ltt-srif-282811.0SSTR4, SSTR5
22srif-142811.0SSTR5, SSTR4
23[125i]tyr10-cst142811.0SSTR5, SSTR4
24pasireotide44 1112.0SSTR5, SST
25[d-tyr8]cyn 1548062811.0SSTR5, SSTR4
26[l-tyr8]cyn 1548062811.0SSTR5, SSTR4
27streptozotocin4411.0SST, GCK, IAPP
28bim 236272811.0SSTR5, SSTR4
29bim 230522811.0SSTR4, SSTR5
30cgp 239962811.0SSTR5, SSTR4
31ke 1082810.9SSTR4, SSTR5
32l-362,8552810.9SSTR4, SSTR5
33[125i]cgp 239962810.9SSTR5, SSTR4
34bim 234542810.9SSTR5, SSTR4
35cst-172810.9SSTR4, SSTR5
36taurodeoxycholate4410.9SCT, SST
37cst-142810.9SSTR5, SSTR4
38glp-1 (7-36)amide4410.9GLP1R, SCT
39metformin44 49 1112.9IAPP, GCK, GLP1R
40lafutidine4410.9SST, SCT
41liraglutide44 28 1112.8IAPP, GLP1R
42exenatide44 1111.8IAPP, GLP1R
43loxiglumide4410.8SST, SCT
44vapreotide28 44 1112.7SSTR5, SST
45ribonucleic acid4410.7GIPR, SSTR4, SSTR5, CASR
46111in-pentetreotide4410.7SSTR5, SST
47sitagliptin44 1111.7IAPP, GLP1R
48proglucagon4410.6GCK, GLP1R
49i-app4410.5IAPP, GCK
50repaglinide44 49 1112.3PAX4, IAPP

GO Terms for genes affiliated with Insulinoma

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16Gene Ontology
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Biological processes related to Insulinoma according to GeneCards/GeneDecks:

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1endocrine pancreas developmentGO:03101811.0PAX4, INSM1, GIPR, IAPP, GCK
2regulation of insulin secretionGO:05079610.9KCNA5, GCK, SSTR5, GLP1R
3response to glucose stimulusGO:00974910.8PTPRN, GIPR, MAFA
4regulation of potassium ion transportGO:04326610.7GCK, KCNA5
5glucose homeostasisGO:04259310.7GCK, WFS1, SSTR5
6negative regulation of cell proliferationGO:00828510.7SST, SSTR4, SSTR5, MEN1
7elevation of cytosolic calcium ion concentrationGO:00720410.7GLP1R, GIPR, GCK
8G-protein coupled receptor signaling pathwayGO:00718610.6CASR, SSTR5, SSTR4, SST
9calcium ion importGO:07050910.3CASR, GCK

Molecular functions related to Insulinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:00517910.7SST, IAPP, SCT
2somatostatin receptor activityGO:00499410.3SSTR4, SSTR5

Products for genes affiliated with Insulinoma

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Sources for Insulinoma

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet