MCID: INS001
MIFTS: 60

Insulinoma

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Insulinoma

MalaCards integrated aliases for Insulinoma:

Name: Insulinoma 12 75 53 59 55 6 44 15 17 72
Islet Cell Adenoma 12 72
Experimental Organism Islet Cell Adenoma Neoplasm 72
Insulin-Producing Tumor of Islet Cells 12
Adenoma Islet Cell 55
Islet Cell Tumor 72

Characteristics:

Orphanet epidemiological data:

59
insulinoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Ireland),1-9/1000000 (Worldwide),1-9/1000000 (Japan); Age of onset: All ages;

Classifications:



External Ids:

Disease Ontology 12 DOID:3892
SNOMED-CT 68 25324008 76345009
MESH via Orphanet 45 D007340
ICD10 via Orphanet 34 E16.8
UMLS via Orphanet 73 C0021670
Orphanet 59 ORPHA97279
UMLS 72 C0021670 C0022134 C0242363 more

Summaries for Insulinoma

NIH Rare Diseases : 53 Insulinoma is a type of pancreatic neuroendocrine tumor (pancreatic NET), which refers to a group of rare tumors that form in the hormone-making cells of the pancreas. Insulinomas, specifically, produce too much insulin, a hormone that reduces the level of sugar in the blood by helping it move into cells. As a result, people with insulinomas generally have very low blood sugar levels which can be associated with anxiety, confusion, hunger, a fast heart rate, and sweating. In severe cases, it can lead to seizures, coma or even death. Ninty percent of insulinomas are benign (noncancerous). In most cases, the underlying cause of insulinoma is unknown. However, people with specific genetic syndromes such as multiple endocrine neoplasia type I, Von Hippel-Lindau syndrome, Neurofibromatosis type 1, and tuberous sclerosis are at risk of insulinomas and other endocrine tumors. Treatment generally includes surgery to remove the tumor.

MalaCards based summary : Insulinoma, also known as islet cell adenoma, is related to insulinomatosis and diabetes mellitus and hypoglycemia. An important gene associated with Insulinoma is MEN1 (Menin 1), and among its related pathways/superpathways are Aldosterone synthesis and secretion and Glucose / Energy Metabolism. The drugs Parathyroid hormone and Cinacalcet have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, pancreas and pancreatic islet, and related phenotypes are hyperhidrosis and seizures

Disease Ontology : 12 A pancreatic cystadenoma that is characterized by the overproduction of insulin.

Wikipedia : 75 An insulinoma is a tumor of the pancreas that is derived from beta cells and secretes insulin. It is a... more...

Related Diseases for Insulinoma

Diseases related to Insulinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 382)
# Related Disease Score Top Affiliating Genes
1 insulinomatosis and diabetes mellitus 33.0 SST SCT INS
2 hypoglycemia 32.0 INS GCK GCG
3 hyperinsulinism 31.8 SST INS GCK
4 hyperinsulinemic hypoglycemia 31.4 SST MEN1 INS GCK
5 multiple endocrine neoplasia, type i 31.4 SST SCT MEN1 INS
6 zollinger-ellison syndrome 31.3 SST SCT MEN1
7 glucagonoma 31.3 SST SCT
8 islet cell tumor 31.2 SST MEN1 INS IAPP
9 gastrinoma 31.0 SST SCT MEN1 INS
10 neuroendocrine tumor 30.9 SSTR2 SST MEN1 INSM1
11 pituitary tumors 30.6 SSTR5 SST MEN1
12 vipoma 30.6 SST SCT
13 duodenal ulcer 30.5 SST SCT INS
14 hyperproinsulinemia 30.3 SCT INS
15 hirata disease 30.3 INS GCG
16 pituitary gland disease 30.3 SST MEN1 INS
17 glucose intolerance 30.2 INS GCK GCG
18 hyperpituitarism 30.1 SST INS
19 pancreatic agenesis 1 30.1 SCT PDX1
20 endocrine gland cancer 30.0 SST MEN1 IAPP GCG
21 dumping syndrome 30.0 SST SCT INS GCG
22 maturity-onset diabetes of the young, type 3 29.9 PDX1 INS GCK
23 carcinoid tumors, intestinal 29.8 SSTR2 SST MEN1
24 pituitary adenoma, prolactin-secreting 29.6 SSTR5 SSTR2 SST MEN1
25 pancreatic cholera 29.5 SST SCT MEN1 GCG
26 small cell cancer of the lung 29.5 SSTR2 SST INSM1
27 pancreatic endocrine carcinoma 29.4 SSTR5 SSTR2 SST INS
28 thyroid carcinoma, familial medullary 29.4 SSTR5 SSTR2 SST MEN1
29 acromegaly 29.1 SSTR5 SSTR2 SST MEN1 INS
30 pancreas disease 29.0 SST SCT INS GLP1R GCG
31 body mass index quantitative trait locus 11 28.7 SLC2A2 INS IAPP GLP1R GCK GCG
32 hyperglycemia 28.6 SST SLC2A2 PDX1 INS IAPP GLP1R
33 maturity-onset diabetes of the young 28.2 SLC2A2 PDX1 INS IAPP GLP1R GCK
34 diabetes mellitus, insulin-dependent 28.1 WFS1 SST PTPRN2 PTPRN INS IAPP
35 diabetes mellitus, noninsulin-dependent 27.5 WFS1 SST SLC2A2 PTPRN PDX1 INS
36 diabetes mellitus 26.3 WFS1 SST SLC2A2 PTPRN2 PTPRN PDX1
37 insulinoma tumor suppressor gene locus 12.4
38 metastatic insulinoma 12.4
39 adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia 12.3
40 pancreatic neuroendocrine tumor 11.6
41 multiple endocrine neoplasia 10.8
42 microinvasive cervical squamous cell carcinoma 10.7 PTPRN INSM1
43 fallopian tube endometrioid adenocarcinoma 10.7 PTPRN INSM1
44 adenoma 10.6
45 pancreatic somatostatinoma 10.6 SST MEN1
46 pancreatic gastrinoma 10.6 SCT MEN1
47 fallopian tube adenocarcinoma 10.6 PTPRN INSM1
48 postgastrectomy syndrome 10.6 SST INS
49 small intestine neuroendocrine neoplasm 10.6 SCT MEN1
50 intestinal neuroendocrine benign tumor 10.6 SCT MEN1

Graphical network of the top 20 diseases related to Insulinoma:



Diseases related to Insulinoma

Symptoms & Phenotypes for Insulinoma

Human phenotypes related to Insulinoma:

59 32 (show all 34)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperhidrosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000975
2 seizures 59 32 hallmark (90%) Very frequent (99-80%) HP:0001250
3 tremor 59 32 hallmark (90%) Very frequent (99-80%) HP:0001337
4 hyperinsulinemic hypoglycemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0000825
5 fasting hyperinsulinemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0008283
6 recurrent hypoglycemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001988
7 palpitations 59 32 hallmark (90%) Very frequent (99-80%) HP:0001962
8 transient global amnesia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010534
9 nonketotic hypoglycemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001958
10 abnormality of the pancreatic islet cells 59 32 hallmark (90%) Very frequent (99-80%) HP:0006476
11 generalized muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0003324
12 zollinger-ellison syndrome 59 32 frequent (33%) Frequent (79-30%) HP:0002044
13 pituitary prolactin cell adenoma 59 32 frequent (33%) Frequent (79-30%) HP:0006767
14 increased body weight 59 32 frequent (33%) Frequent (79-30%) HP:0004324
15 polyphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002591
16 reactive hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0012051
17 fluctuations in consciousness 59 32 frequent (33%) Frequent (79-30%) HP:0007159
18 primary hyperparathyroidism 59 32 frequent (33%) Frequent (79-30%) HP:0008200
19 fatigue 59 32 occasional (7.5%) Occasional (29-5%) HP:0012378
20 abnormality of vision 59 32 occasional (7.5%) Occasional (29-5%) HP:0000504
21 hearing abnormality 59 32 occasional (7.5%) Occasional (29-5%) HP:0000364
22 anxiety 59 32 occasional (7.5%) Occasional (29-5%) HP:0000739
23 paresthesia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003401
24 neuroendocrine neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0100634
25 coma 59 32 occasional (7.5%) Occasional (29-5%) HP:0001259
26 insomnia 59 32 occasional (7.5%) Occasional (29-5%) HP:0100785
27 lethargy 59 32 occasional (7.5%) Occasional (29-5%) HP:0001254
28 neoplasm of the adrenal gland 59 32 occasional (7.5%) Occasional (29-5%) HP:0100631
29 abnormal rapid eye movement sleep 59 32 occasional (7.5%) Occasional (29-5%) HP:0002494
30 abnormality of pain sensation 59 32 occasional (7.5%) Occasional (29-5%) HP:0010832
31 behavioral abnormality 59 Frequent (79-30%)
32 reduced consciousness/confusion 59 Frequent (79-30%)
33 hyperinsulinemia 59 Very frequent (99-80%)
34 abnormality of higher mental function 59 Occasional (29-5%)

MGI Mouse Phenotypes related to Insulinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.85 GCK GLP1R INS INSM1 MEN1 PDX1
2 digestive/alimentary MP:0005381 9.8 INS INSM1 MEN1 PDX1 SST SSTR2
3 growth/size/body region MP:0005378 9.7 GCK GLP1R IAPP INS INSM1 MEN1
4 homeostasis/metabolism MP:0005376 9.5 GCK GLP1R IAPP INS INSM1 KCNA5

Drugs & Therapeutics for Insulinoma

Drugs for Insulinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 168)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Parathyroid hormone Approved, Investigational Phase 3 9002-64-6
2
Cinacalcet Approved Phase 3 226256-56-0 156419
3
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
4
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616 46835353
5
Everolimus Approved Phase 3 159351-69-6 6442177 70789204
6
lanreotide Approved Phase 2, Phase 3 108736-35-2
7
Sunitinib Approved, Investigational Phase 3 557795-19-4, 341031-54-7 5329102
8
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
9 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
10 Hormone Antagonists Phase 3
11 Hormones Phase 3
12 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
13 Central Nervous System Depressants Phase 3
14 Liver Extracts Phase 3
15 Calcium, Dietary Phase 3
16 Cola Phase 3
17 Peripheral Nervous System Agents Phase 3
18 Calcimimetic Agents Phase 3
19 Analgesics Phase 3
20 Anesthetics, Intravenous Phase 3
21 Narcotics Phase 3
22 Analgesics, Opioid Phase 3
23 Adjuvants, Anesthesia Phase 3
24 Anesthetics, General Phase 3
25 Anesthetics Phase 3
26 Angiopeptin Phase 2, Phase 3
27 Angiogenesis Inhibitors Phase 3
28 Angiogenesis Modulating Agents Phase 3
29
Tegafur Approved, Investigational Phase 2 17902-23-7 5386
30
Metformin Approved Phase 2 657-24-9 4091 14219
31
Exenatide Approved, Investigational Phase 1, Phase 2 141758-74-9 15991534
32
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
33
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
34
Nicotinamide Approved, Investigational Phase 2 98-92-0 936
35
Palbociclib Approved, Investigational Phase 2 571190-30-2 5330286 11431660 5005498
36
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
37
Melphalan Approved Phase 2 148-82-3 460612 4053
38
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
39
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
40
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
41
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
42
Glycerol Approved, Investigational Phase 2 56-81-5 753
43
Zinc Approved, Investigational Phase 2 7440-66-6 32051
44
Fluorouracil Approved Phase 2 51-21-8 3385
45
Pancrelipase Approved, Investigational Phase 2 53608-75-6
46
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
47
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
48
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
49
Bevacizumab Approved, Investigational Phase 2 216974-75-3
50
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447

Interventional clinical trials:

(show top 50) (show all 139)
# Name Status NCT ID Phase Drugs
1 Clinical Effectiveness of Serum Chromogranin A (CgA) Levels on Diagnostic Relevance, Response After Surgical Resection and Recurrence of Pancreatic Endocrine Tumors (PET) Unknown status NCT02759718 Phase 4
2 A SINGLE-ARM OPEN-LABEL INTERNATIONAL MULTI-CENTER STUDY OF THE EFFICACY AND SAFETY OF SUNITINIB MALATE (SU011248, SUTENT (REGISTERED)) IN PATIENTS WITH PROGRESSIVE ADVANCED METASTATIC WELL-DIFFERENTIATED UNRESECTABLE PANCREATIC NEUROENDOCRINE TUMORS Completed NCT01525550 Phase 4 sunitinib
3 Phase IV, Open-label, Multi-center, Single-arm Study of the Safety and Efficacy of Everolimus (Afinitor) in Adult Patients With Local Advanced or Metastatic, Well Differentiated Progressive Pancreatic Neuroendocrine Tumors (pNET) in China. Active, not recruiting NCT02842749 Phase 4 everolimus
4 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Unknown status NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
5 Phase III, Randomised, Double-blind, Stratified Comparative, Placebo Controlled, Parallel Group, Multi-centre Study to Assess the Effect of Deep Subcutaneous Injections of Lanreotide Autogel 120mg Administered Every 28 Days on Tumour Progression Free Survival in Patients With Non-functioning Entero-pancreatic Endocrine Tumour Completed NCT00353496 Phase 3 lanreotide (Autogel formulation);Placebo
6 Open Label Extension Study of Lanreotide Autogel 120 mg in Patients With Non-functioning Entero-pancreatic Endocrine Tumour Completed NCT00842348 Phase 3 lanreotide (Autogel formulation)
7 Cinacalcet Actions in Familial Primary Hyperparathyroidism Completed NCT00325104 Phase 3
8 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
9 A EUROPEAN, MULTICENTRE, PHASE II/III RANDOMISED DOUBLE-BLIND, PLACEBO CONTROLLED STUDY EVALUATING LANREOTIDE AS MAINTENANCE THERAPY IN PATIENTS WITH NON-RESECTABLE DUODENO-PANCREATIC NEUROENDOCRINE TUMOURS AFTER FIRST-LINE TREATMENT Recruiting NCT02288377 Phase 2, Phase 3 lanreotide;Placebo
10 A Randomized, Double-blind, Multi-center Phase III Clinical Study to Assess the Efficacy and Safety of Surufatinib Compared to Placebo in Patients With Advanced Pancreatic Neuroendocrine Tumors Recruiting NCT02589821 Phase 3 Surufatinib
11 Randomized, Double-Blinded Phase III Study of CABozantinib Versus Placebo IN Patients With Advanced NEuroendocrine Tumors After Progression on Everolimus (CABINET) Recruiting NCT03375320 Phase 3 Cabozantinib S-malate
12 Non-functioning Pancreatic Neuroendocrine Tumors (NF-pNETs) in Multiple Endocrine Neoplasia Type 1 (MEN1) Treated With Somatostatin Analogs (SA) Versus NO Treatment - a Prospective, Randomized, Controlled Multicenter Study Not yet recruiting NCT02705651 Phase 3 Somatostatin-Analog
13 A Phase III Randomized, Double-Blind Study Of Sunitinib (SU011248, SUTENT) Versus Placebo In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Islet Cell Tumors Terminated NCT00428597 Phase 3 sunitinib malate;Placebo
14 68Ga-DOTATATE PET Scan Imaging in Patients With Neuroendocrine Tumors Unknown status NCT02038738 Phase 1, Phase 2 68Ga-DOTATATE will be given in tracer doses and injected intravenously to image tumors by Positron Emission Tomography.
15 A Phase II Randomized,Controlled,Open Label,Multicentre Study of Tegafur Combined With Temozolomide Versus Tegafur Combined With Temozolomide and Thalidomide in Subjects With Advanced Pancreatic Neuroendocrine Tumor Unknown status NCT03204019 Phase 2 Tegafur and Temozolomide;Tegafur and Temozolomide combined with Thalidomide
16 Activity and Safety of Everolimus in Combination With Octreotide LAR and Metformin in Patients With Advanced Pancreatic Well-differentiated Neuroendocrine Tumors (pWDNETs): a Phase II, Open, Monocentric, Prospective Study Unknown status NCT02294006 Phase 2 Everolimus plus Octreotide LAR plus Metformin
17 68Ga-NODAGA-exendin-4 PET/CT in Patients With AHH - a Prospective Comparative Evaluation of Preoperative Imaging Unknown status NCT03189953 Phase 1, Phase 2
18 Evaluation of Chemotherapy With Streptozotocin Combined With 5-Fluorouracil and Adriamycin in Patients With Zollinger-Ellison Syndrome and Metastatic Non-Beta-Islet Cell Neoplasm Completed NCT00001165 Phase 2 combined chemotherapy with streptozotocin, 5-fluorouracil, and doxorubicin
19 Evaluation of Treatment With Interferon, Octreotide, or Their Combination in Patients With Zollinger-Ellison Syndrome and Progressive Metastatic Non-B Islet Cell Neoplasm Completed NCT00001228 Phase 2 Interferon
20 A Randomised Phase II Study Comparing Capecitabine Plus Streptozocin With or Without Cisplatin Chemotherapy as Treatment for Unresectable or Metastatic Neuroendocrine Tumors Completed NCT00602082 Phase 2 capecitabine;cisplatin;streptozocin
21 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
22 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
23 Study of First-Line Therapy Comprising Leucovorin Calcium, Fluorouracil, and Irinotecan (FOLFIRI) in Patients With Progressive Locally Advanced or Metastatic Duodenal-Pancreatic Endocrine Tumors Completed NCT00416767 Phase 2 fluorouracil;irinotecan hydrochloride;leucovorin calcium
24 A Phase II Study Of Sunitinib In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Neuroendocrine Tumors Completed NCT01121562 Phase 2 Sunitinib
25 A Phase II Clinical and Biologic Study of AMG 706 and Octreotide in Patients With Low-Grade Neuroendocrine Tumors Completed NCT00427349 Phase 2 AMG 706;octreotide
26 A Phase II Trial to Assess the Activity and Safety of Palbociclib in Patients With Well and Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors (pNET) Completed NCT02806648 Phase 2 Palbociclib
27 A Multicenter, Two Stage, Phase II Study, Evaluating the Efficacy of Oral BEZ235 Plus Best Supportive Care (BSC) Versus Placebo Plus BSC in the Treatment of Patients With Advanced Pancreatic Neuroendocrine Tumors (pNET) After Failure of mTOR Inhibitor Therapy. Completed NCT01658436 Phase 2 BEZ235 (Stage 1)
28 Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization for Metastatic Gastrointestinal Neuroendocrine Tumors Completed NCT00434109 Phase 2 Sunitinib malate
29 A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors Completed NCT01024387 Phase 2 AMG 479
30 A Phase II Study of Isolated Hepatic Perfusion (IHP) With Melphalan for Metastatic Unresectable Cancers of the Liver Completed NCT00019786 Phase 2 isolated perfusion;melphalan
31 Perfusion CT as Predictive Biomarker in a Phase II Study of Ziv-Aflibercept in Patients With Advanced Pancreatic Neuroendocrine Tumors Completed NCT02101918 Phase 2
32 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
33 Accuracy and Clinical Impact of 68-Ga-labeled Octreotide Analogues PET in Diagnosis and Staging of Duodenal-pancreatic Neuroendocrine Tumours; Proposal of a Multicenter, Prospective Clinical Trial Completed NCT01673906 Phase 2 Diagnostic work up
34 An Angiogenic Study in Patients With Well/Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors Treated With Everolimus Completed NCT02305810 Phase 2 Everolimus 10 mg daily
35 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
36 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
37 A Phase 2 Trial of Temsirolimus and Bevacizumab in Patients With Endometrial, Ovarian, Hepatocellular Carcinoma, Carcinoid or Islet Cell Cancer Completed NCT01010126 Phase 2 Temsirolimus
38 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
39 A Phase II Study Of Hepatic Arterial Infusion Of Melphalan With Venous Filtration Via Peripheral Hepatic Perfusion (PHP) For Unresectable Primary And Metastatic Cancers Of The Liver Completed NCT00096083 Phase 2 isolated perfusion;melphalan
40 A Phase II, Single-Arm Open-Label Study of the Combination of Atezolizumab and Bevacizumab in Rare Solid Tumors Recruiting NCT03074513 Phase 2 Atezolizumab
41 A Phase II, Open-label Study of CVM-1118 Administered Orally to Patients With Advanced Neuroendocrine Tumors Recruiting NCT03600233 Phase 2 CVM-1118
42 Phase II Study to Evaluate Efficacy of Rechallenge With Sunitinib in Patients With Metastatic Pancreatic Neuroendocrine Tumor (pNETs) Well Differentiated G1/2 Advanced or Metastatic Who Previously Failed to Sunitinib. Recruiting NCT02713763 Phase 2 Sunitinib
43 Peptide Receptor Radionuclide Therapy (PRRT) With Radiolabelled Somatostatin Analogue 177Lu-DOTATATE in Advanced Gastro-entero Pancreatic Neuroendocrine Tumors, 18F-2-fluoro-2-deoxy-D-glucose (FDG)-PET Negative Patients: a Prospective Phase II Randomized Study Recruiting NCT02489604 Phase 2 177Lu-DOTATATE 25.9 GBq activity;177Lu-DOTATATE 18.5 GBq activity
44 177Lutethium - Peptide Receptor Radionuclide Therapy (Lu-PRRT) Plus Capecitabine Versus Lu-PRRT in FDG Positive, Gastro-entero-pancreatic Neuroendocrine Tumors: a Randomized Phase II Study. Recruiting NCT02736448 Phase 2 Capecitabine;Lu-PRRT;SS-LAR
45 Capecitabine ON Temozolomide Radionuclide Therapy Octreotate Lutetium-177 NeuroEndocrine Tumours Study Recruiting NCT02358356 Phase 2 octreotate;Capecitabine;Temozolomide
46 A Phase II Study of Capecitabine, Temozolomide and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 capecitabine;temozolomide
47 A Randomized Phase II Study of Temozolomide or Temozolomide and Capecitabine in Patients With Advanced Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01824875 Phase 2 temozolomide;capecitabine
48 A Phase I/II Study of X-82, an Oral Anti-VEGFR Tyrosine Kinase Inhibitor, With Everolimus for Patients With Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01784861 Phase 1, Phase 2 X-82;Everolimus
49 Phase II Multicenter Single-arm Study Evaluating the Safety and Efficacy of Everolimus as a First-line Treatment in Newly-diagnosed Patients With Advanced GI Neuroendocrine Tumors. Active, not recruiting NCT01648465 Phase 2 Everolimus
50 Phase I/II Study of RAD001 in Combination With Temozolomide in Patients With Advanced Pancreatic Neuroendocrine Tumors Active, not recruiting NCT00576680 Phase 1, Phase 2 RAD001;Temozolomide

Search NIH Clinical Center for Insulinoma

Cochrane evidence based reviews: insulinoma

Genetic Tests for Insulinoma

Anatomical Context for Insulinoma

MalaCards organs/tissues related to Insulinoma:

41
Pancreas, Pancreatic Islet, Liver, Testes, T Cells, Brain, Heart
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Insulinoma:
# Tissue Anatomical CompartmentCell Relevance
1 Pancreas Islets of Langerhans Mature Beta Cells Affected by disease

Publications for Insulinoma

Articles related to Insulinoma:

(show top 50) (show all 5596)
# Title Authors PMID Year
1
IFN-gamma and IL-10 islet-antigen-specific T cell responses in autoantibody-negative first-degree relatives of patients with type 1 diabetes. 9 38
20369219 2010
2
Role of JNK activation in pancreatic beta-cell death by streptozotocin. 9 38
20176078 2010
3
Ghrelin inhibits insulin secretion through the AMPK-UCP2 pathway in beta cells. 9 38
20206170 2010
4
Expression of TAU in insulin-secreting cells and its interaction with the calcium-binding protein secretagogin. 9 38
20061514 2010
5
Biological activity of EXf, a peptide analogue of exendin-4. 9 38
19919832 2010
6
Evidence for molecular mimicry between human T cell epitopes in rotavirus and pancreatic islet autoantigens. 9 38
20083660 2010
7
Pancreatic duodenal homeobox 1 protein is a novel beta-cell-specific autoantigen for type I diabetes. 9 38
19901909 2010
8
Naturally occurring glucokinase mutations are associated with defects in posttranslational S-nitrosylation. 9 38
19934346 2010
9
Glucagon-like peptide-1 receptor imaging for localization of insulinomas. 9 38
19820010 2009
10
Autophagy regulates pancreatic beta cell death in response to Pdx1 deficiency and nutrient deprivation. 9 38
19654319 2009
11
Successful control of intractable hypoglycemia using rapamycin in an 86-year-old man with a pancreatic insulin-secreting islet cell tumor and metastases. 9 38
19567519 2009
12
Evaluation of endocrine tests. D: the prolonged fasting test for insulinoma. 9 38
19687521 2009
13
[Hyperinsulinism in infancy and childhood: when an insulin level is not always enough]. 9 38
19411227 2009
14
A new orally available glucagon-like peptide-1 receptor agonist, biotinylated exendin-4, displays improved hypoglycemic effects in db/db mice. 9 38
18977255 2009
15
Application of the intravenous glucose tolerance test and the minimal model to patients with insulinoma: insulin sensitivity (Si) and glucose effectiveness (Sg) before and after surgical excision. 9 38
18445139 2009
16
Expression and function of IA-2 family proteins, unique neuroendocrine-specific protein-tyrosine phosphatases. 9 38
19550073 2009
17
Early loss of mammalian target of rapamycin complex 1 (mTORC1) signalling and reduction in cell size during dominant-negative suppression of hepatic nuclear factor 1-alpha (HNF1A) function in INS-1 insulinoma cells. 9 38
18949455 2009
18
Weight gain in early life predicts risk of islet autoimmunity in children with a first-degree relative with type 1 diabetes. 9 38
18835948 2009
19
Autoantibodies to GAD65 and IA-2 in canine diabetes mellitus. 9 38
18706702 2008
20
MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells. 9 38
18775714 2008
21
Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. 9 38
18692019 2008
22
Expression of HNF4alpha variants in pancreatic islets and Ins-1 beta cells. 9 38
18561282 2008
23
Insulinoma cell calcium-sensing receptor influences insulin secretion in a case with concurrent familial hypocalciuric hypercalcemia and malignant metastatic insulinoma. 9 38
18430790 2008
24
Differentiation of insulin secretion patterns in insulinoma. 9 38
18259806 2008
25
Mice with beta cell overexpression of glycogen synthase kinase-3beta have reduced beta cell mass and proliferation. 9 38
18219478 2008
26
Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. 9 38
18156285 2008
27
In vitro study of using calcium phosphate cement as immunoisolative device to enclose insulinoma/agarose microspheres as bioartificial pancreas. 9 38
17514757 2007
28
Expression and functional consequences of oestrogen and progesterone receptors in human insulinomas. 9 38
18045959 2007
29
Regulation of cAMP dynamics by Ca2+ and G protein-coupled receptors in the pancreatic beta-cell: a computational approach. 9 38
17928534 2007
30
Intra-operative quick insulin assay to confirm complete resection of insulinomas guided by selective arterial calcium injection (SACI). 9 38
17294212 2007
31
Rapid intraoperative insulin assay: a novel method to differentiate insulinoma from nesidioblastosis in the pediatric patient. 9 38
17958895 2007
32
[A 64-year-old female patient with recurring hypoglycaemia. Difficult aspects of diagnosis]. 9 38
17704900 2007
33
Assessment of suspected insulinoma by 48-hour fasting test: a retrospective monocentric study of 23 cases. 9 38
17611903 2007
34
The transcription factor PAX4 acts as a survival gene in INS-1E insulinoma cells. 9 38
17260022 2007
35
[Lys40(Ahx-DTPA-111In)NH2]-Exendin-4 is a highly efficient radiotherapeutic for glucagon-like peptide-1 receptor-targeted therapy for insulinoma. 9 38
17575235 2007
36
Diagnosis and minimally invasive resection of an insulinoma: report of an unusual case and review of the literature. 9 38
17521012 2007
37
Biochemistry of neuroendocrine tumours. 9 38
17382264 2007
38
Insulin responses to selective arterial calcium infusion under hyperinsulinemic euglycemic glucose clamps: case studies in adult nesidioblastosis and childhood insulinoma. 9 38
17053293 2007
39
A case of secretin-responsive insulinoma with low serum C-peptide levels. 9 38
17146148 2007
40
Two conserved domains in PCIF1 mediate interaction with pancreatic transcription factor PDX-1. 9 38
17126328 2006
41
Trefoil factors are expressed in human and rat endocrine pancreas: differential regulation by growth hormone. 9 38
16973727 2006
42
Effects of fluoroquinolones on HERG channels and on pancreatic beta-cell ATP-sensitive K+ channels. 9 38
17027138 2006
43
Caveolin-1 regulates cellular trafficking and function of the glucagon-like Peptide 1 receptor. 9 38
16931572 2006
44
The PDX1 homeodomain transcription factor negatively regulates the pancreatic ductal cell-specific keratin 19 promoter. 9 38
17056599 2006
45
Spontaneous hypoglycaemia in the presence of both anti-insulin antibody and anti-insulin receptor antibody. 9 38
17296513 2006
46
MicroRNA expression abnormalities in pancreatic endocrine and acinar tumors are associated with distinctive pathologic features and clinical behavior. 88
16966691 2006
47
Normal but increasing hemoglobin A1c levels predict progression from islet autoimmunity to overt type 1 diabetes: Diabetes Autoimmunity Study in the Young (DAISY). 9 38
17054445 2006
48
Sp1/Sp3 binding is associated with cell-specific expression of the glucose-dependent insulinotropic polypeptide receptor gene. 9 38
16403775 2006
49
Hypercalcitoninemia in a patient with a recurrent goitre and insulinoma: a case report. 9 38
16804794 2006
50
Downregulation of protein kinase B/Akt-1 mediates INS-1 insulinoma cell apoptosis induced by dominant-negative suppression of hepatocyte nuclear factor-1alpha function. 9 38
16440211 2006

Variations for Insulinoma

ClinVar genetic disease variations for Insulinoma:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 MEN1 NM_000244.3(MEN1): c.371_372del (p.Val124fs) deletion Pathogenic rs1555166368 11:64577210-64577211 11:64809738-64809739

Expression for Insulinoma

Search GEO for disease gene expression data for Insulinoma.

Pathways for Insulinoma

Pathways related to Insulinoma according to GeneCards Suite gene sharing:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.5 SLC2A2 PDX1 INS GLP1R GCK GCG
2 12.29 WFS1 PDX1 INS GCG
3
Show member pathways
12.13 SCT IAPP GLP1R GCG
4
Show member pathways
12.07 SLC2A2 INS GLP1R GCG
5 11.83 SCT IAPP GLP1R GCG
6 11.81 SLC2A2 PDX1 INS
7
Show member pathways
11.72 SLC2A2 PDX1 INS GCK
8 11.66 SLC2A2 GCK GCG
9
Show member pathways
11.44 SLC2A2 PDX1 INS GCK
10
Show member pathways
11.1 SLC2A2 PDX1 INSM1 INS IAPP GCK
11 10.9 SLC2A2 PDX1 INS GCK
12 10.68 SLC2A2 INS

GO Terms for Insulinoma

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

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum lumen GO:0005788 9.02 WFS1 PTPRN2 MEN1 INS GCG

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.92 SSTR5 SSTR2 SST SCT INS IAPP
2 cellular protein metabolic process GO:0044267 9.81 WFS1 MEN1 INS IAPP
3 negative regulation of cell proliferation GO:0008285 9.73 SSTR5 SSTR2 SST PDX1 MEN1 INSM1
4 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.71 IAPP GLP1R GCG
5 glucose metabolic process GO:0006006 9.67 PDX1 INS GCK
6 cellular response to glucocorticoid stimulus GO:0071385 9.57 SSTR5 SSTR2
7 negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway GO:1902236 9.56 WFS1 PDX1
8 positive regulation of glycogen biosynthetic process GO:0045725 9.55 INS GCK
9 glucose homeostasis GO:0042593 9.55 WFS1 SSTR5 PDX1 INS GCK
10 negative regulation of gluconeogenesis GO:0045721 9.52 INS GCK
11 insulin secretion involved in cellular response to glucose stimulus GO:0035773 9.48 PTPRN2 PTPRN
12 negative regulation of type B pancreatic cell apoptotic process GO:2000675 9.46 WFS1 PDX1
13 somatostatin signaling pathway GO:0038170 9.43 SSTR5 SSTR2
14 regulation of potassium ion transport GO:0043266 9.4 KCNA5 GCK
15 detection of glucose GO:0051594 9.37 PDX1 GCK
16 type B pancreatic cell differentiation GO:0003309 9.13 PDX1 MEN1 INSM1
17 regulation of insulin secretion GO:0050796 9.1 SSTR5 SLC2A2 KCNA5 GLP1R GCK GCG

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

# Name GO ID Score Top Affiliating Genes
1 neuropeptide binding GO:0042923 9.16 SSTR5 SSTR2
2 hormone activity GO:0005179 9.02 SST SCT INS IAPP GCG
3 somatostatin receptor activity GO:0004994 8.96 SSTR5 SSTR2

Sources for Insulinoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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