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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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:

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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, B cells, Brain, Lung, Lymph node, Retina, Appendix, Bone, Hypothalamus, 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:000537611.3KCNA5, PTPRN, INSM1, GLP1R, GIPR, MAFA
2MP:000537811.0MAFA, SST, SSTR5, IAPP, WFS1, MEN1
3MP:000537910.8GCK, CASR, MEN1, WFS1, PTPRN, PAX4

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
Surgery versus snacking as treatment of insulinoma. (24388037)
2014
2
Pancreatic insulinoma combined with glucagon positive cell: A case report. (23556064)
2013
3
Therapeutic effect of sunitinib malate and its influence on blood glucose concentrations in a patient with metastatic insulinoma. (23573816)
2013
4
Ectopic duodenal insulinoma: a very rare and challenging tumor type. Description of a case and review of the literature. (24006218)
2013
5
Efficacy of everolimus in patients with metastatic insulinoma and refractory hypoglycemia. (23392213)
2013
6
A case of inoperable malignant insulinoma with resistant hypoglycemia who experienced the most significant clinical improvement with everolimus. (23738155)
2013
7
Indispensable utility of intra-arterial calcium stimulation and hepatic venous sampling in a 7mm insulinoma with poor imaging localization. (22803305)
2012
8
Dietary zinc reduction, pyruvate supplementation, or zinc transporter 5 knockout attenuates I^-cell death in nonobese diabetic mice, islets, and insulinoma cells. (23096014)
2012
9
Mediation of glucolipotoxicity in INS-1 rat insulinoma cells by small heterodimer partner interacting leucine zipper protein (SMILE). (22387546)
2012
10
Everolimus induces rapid plasma glucose normalization in insulinoma patients by effects on tumor as well as normal tissues. (21482586)
2011
11
Effects of 1-methylnicotinamide and its metabolite N-methyl-2-pyridone-5-carboxamide on streptozotocin-induced toxicity in murine insulinoma MIN6 cell line. (21403919)
2011
12
Paroxysmal dystonic choreoathetosis with symptomatic seizures secondary to hypoglycemia caused by insulinoma. (22346027)
2011
13
Hemoglobin regulates the metabolic and synthetic function of rat insulinoma cells cultured in a hollow fiber bioreactor. (20552668)
2010
14
Pancreatic insulinoma: a surgical experience. (19629581)
2009
15
Radiofrequency ablation of solitary pancreatic insulinoma in a patient with episodes of severe hypoglycemia. (19685572)
2009
16
Embolization as an alternative treatment of insulinoma in a patient with multiple endocrine neoplasia type 1 syndrome. (19184198)
2009
17
Insulinoma and pregnancy. (19111295)
2009
18
Clearance of acanthosis nigricans associated with insulinoma following surgical resection. (18669553)
2008
19
Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. (18692019)
2008
20
Successful endoscopic ultrasound-guided ethanol ablation of a sporadic insulinoma. (19198582)
2008
21
Constitutively active Akt1 expression in mouse pancreas requires S6 kinase 1 for insulinoma formation. (18846252)
2008
22
Rapid intraoperative insulin assay: a novel method to differentiate insulinoma from nesidioblastosis in the pediatric patient. (17958895)
2007
23
Engineering and expression of the intracellular domain of insulinoma-associated tyrosine phosphatase (IA-2ic), a type 1 diabetes autoantigen, in plants. (17103024)
2007
24
Melatonin stimulates inositol-1,4,5-trisphosphate and Ca2+ release from INS1 insulinoma cells. (16150114)
2005
25
Successful long-term treatment with once-daily injection of low-dose octreotide in an aged patient with insulinoma. (16284444)
2005
26
A synthetic polymer, poly(2-methacryloyloxyethyl phosphorylcholine-co-n-stearyl methacrylate), stimulates insulin release from RINm5F insulinoma cells. (15502370)
2004
27
Cytological and immunocytochemical characterization of the insulin secreting insulinoma cell line RINm5F. (15125025)
2004
28
Genetically-driven or supposed genetic-related insulinomas in adults: validation of the surgical strategy proposed by the A.F.C.E./G.E.N.E.M]. (15247875)
2004
29
Expression and function of the calcium-sensing receptor in pancreatic islets and insulinoma cells. (12604917)
2003
30
Insulinoma with hyperproinsulinemia: a two cases report]. (12193882)
2002
31
Adult hyperinsulinemic hypoglycemia not caused by an insulinoma: a report of two cases. (10881742)
2000
32
Molecular basis for the inactivation of Ca2+- and voltage-dependent BK channels in adrenal chromaffin cells and rat insulinoma tumor cells. (10377337)
1999
33
Beta-cell gene expression and functional characterisation of the human insulinoma cell line CM. (10194529)
1999
34
Glucose- and phorbol ester-induced insulin secretion in human insulinoma cells--association with protein kinase C activation. (9844735)
1998
35
A hyperinsulinaemic, sequentially eu- and hypoglycaemic clamp test to characterize autonomous insulin secretion in patients with insulinoma. (9061303)
1997
36
Proteins spontaneously released by rat insulinoma (RIN) cells are anchored on cell membrane by a glycosyl-phosphatidyl-inositol link and inhibit increased RIN cell adhesion of lymphocytes from type 1 diabetic patients and non-obese diabetic mice in vitro. (8985653)
1996
37
Eicosapentaenoic acid (C20:5) augments glucose-induced insulin secretion from beta-TC3 insulinoma cells. (8884846)
1996
38
Chronic primary hyperinsulinaemia is associated with altered insulin receptor mRNA splicing in muscle of patients with insulinoma. (8635675)
1996
39
HLA-DR-restricted T cell lines from newly diagnosed type 1 diabetic patients specific for insulinoma and normal islet beta cell proteins: lack of reactivity to glutamic acid decarboxylase. (7554382)
1995
40
Detection of the human glucagon-like peptide 1(7-36) amide receptor on insulinoma-derived cell membranes. (8112494)
1994
41
Proinsulin by immunochemiluminometric assay for the diagnosis of insulinoma. (8175958)
1994
42
Surgical treatment of insulinoma. (8044542)
1994
43
Relapse of medically treated insulinoma following the development of thyrotoxicosis: an assessment of potential mechanisms. (8054864)
1994
44
Intraarterial calcium stimulation test for detection of insulinomas. (8362527)
1993
45
Rat insulinoma cells express both a 115-kDa growth hormone receptor and a 95-kDa prolactin receptor structurally related to the hepatic receptors. (1692834)
1990
46
Immunocytochemical staining for human chorionic gonadotropin subunits does not predict malignancy in insulinomas. (1689098)
1990
47
Lack of association between blood pressure and insulin in patients with insulinoma. (2163424)
1990
48
Introduction of exogenous growth hormone receptors augments growth hormone-responsive insulin biosynthesis in rat insulinoma cells. (2205855)
1990
49
Forced diuresis to reduce nephrotoxicity of streptozotocin in the treatment of advanced metastatic insulinoma. (3034373)
1987
50
Malignant metastatic insulinoma treated with streptozotocin. (170028)
1975

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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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
1glucose4411.5GCK, CASR, SCT, IAPP, SST, MAFA
2calcium44 49 11 2414.3KCNA5, GCK, CASR, SCT, MEN1, WFS1
3c-peptide4411.3PTPRN, GCK, SCT, IAPP, SST, GLP1R
4adenylate4411.3GCK, CASR, SCT, IAPP, SST, GLP1R
5acth4411.2CASR, SCT, SSTR5, SST, GIPR
6potassium44 11 2413.2KCNA5, GCK, CASR, SCT, GLP1R
7octreotide44 59 28 1114.2SCT, SSTR5, SSTR4, SST
8arginine4411.1GCK, CASR, SCT, IAPP, SST, PTPRN
9(1r,1s,3r/1r,1r,3s)-l-054,2645911.1SSTR5, SSTR4, SST
10cyclosomatostatin5911.1SSTR5, SSTR4, SST
11cyn 1548065911.1SSTR5, SSTR4, SST
12somatostatin59 2412.1SST, SSTR4, SSTR5
13incretin4411.1GLP1R, IAPP, GCK
14oxyntomodulin44 5912.1GLP1R, SST, SCT
15lanreotide44 2812.1SSTR5, SSTR4, SST
16sulfonylurea4411.1GCK, IAPP, GLP1R
17[125i]tyr11-srif-142811.0SSTR4, SSTR5
18prl-29152811.0SSTR4, SSTR5
19des-aa5-[d-trp8]srif2811.0SSTR4, SSTR5
20bim 236272811.0SSTR4, SSTR5
21l-362,8552811.0SSTR5, SSTR4
22ke 1082811.0SSTR5, SSTR4
23cgp 239962811.0SSTR5, SSTR4
24bim 234542811.0SSTR4, SSTR5
25[l-tyr8]cyn 1548062811.0SSTR4, SSTR5
26[d-tyr8]cyn 1548062811.0SSTR4, SSTR5
27streptozotocin4411.0SST, IAPP, GCK
28pasireotide44 1112.0SST, SSTR5
29bim 230522811.0SSTR5, SSTR4
30taurodeoxycholate4411.0SST, SCT
31[125i]ltt-srif-282810.9SSTR4, SSTR5
32[125i]tyr10-cst142810.9SSTR4, SSTR5
33srif-142810.9SSTR4, SSTR5
34srif-282810.9SSTR4, SSTR5
35[125i]cgp 239962810.9SSTR5, SSTR4
36cst-172810.9SSTR5, SSTR4
37cst-142810.9SSTR5, SSTR4
38glp-1 (7-36)amide4410.9SCT, GLP1R
39metformin44 49 1112.9GLP1R, IAPP, GCK
40lafutidine4410.9SCT, SST
41liraglutide44 28 1112.8IAPP, GLP1R
42exenatide44 1111.8IAPP, GLP1R
43loxiglumide4410.8SCT, SST
44vapreotide28 44 1112.7SSTR5, SST
45ribonucleic acid4410.7CASR, SSTR5, SSTR4, GIPR
46111in-pentetreotide4410.7SSTR5, SST
47sitagliptin44 1111.7GLP1R, IAPP
48proglucagon4410.6GLP1R, GCK
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:03101811.0INSM1, PAX4, GIPR, IAPP, GCK
2regulation of insulin secretionGO:05079610.9SSTR5, GLP1R, KCNA5, GCK
3response to glucose stimulusGO:00974910.8PTPRN, GIPR, MAFA
4regulation of potassium ion transportGO:04326610.7KCNA5, GCK
5glucose homeostasisGO:04259310.7WFS1, GCK, SSTR5
6negative regulation of cell proliferationGO:00828510.7SST, SSTR4, SSTR5, MEN1
7elevation of cytosolic calcium ion concentrationGO:00720410.7GCK, GIPR, GLP1R
8G-protein coupled receptor signaling pathwayGO:00718610.6SST, SSTR4, SSTR5, CASR
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