MCID: INS001
MIFTS: 59

Insulinoma

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

Aliases & Classifications for Insulinoma

MalaCards integrated aliases for Insulinoma:

Name: Insulinoma 12 73 20 58 54 6 44 15 17 70
Islet Cell Adenoma 12 70
Experimental Organism Islet Cell Adenoma Neoplasm 70
Insulin-Producing Tumor of Islet Cells 12
Adenoma Islet Cell 54
Islet Cell Tumor 70

Characteristics:

Orphanet epidemiological data:

58
insulinoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Ireland),1-9/1000000 (Worldwide),1-9/1000000 (Japan); Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


Summaries for Insulinoma

GARD : 20 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 hypoglycemia and hyperinsulinism. 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 Sunitinib and Protein Kinase Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, pancreatic islet and pituitary, and related phenotypes are hyperhidrosis and tremor

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

Wikipedia : 73 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 418)
# Related Disease Score Top Affiliating Genes
1 hypoglycemia 31.5 SST SLC2A2 INS IAPP GLP1R GCK
2 hyperinsulinism 31.4 SST INS GCK GCG ABCC8
3 multiple endocrine neoplasia 31.3 SST SCT MEN1 GAST
4 neuroendocrine tumor 31.0 SST SCT MEN1 INSM1 GCG GAST
5 hyperinsulinemic hypoglycemia 30.9 SST PDX1 MEN1 INS GCK GCG
6 glucagonoma 30.9 SST SCT GAST
7 zollinger-ellison syndrome 30.9 SST SCT MEN1 GAST
8 islet cell tumor 30.8 SST SCT MEN1 INS IAPP GCG
9 neuroendocrine carcinoma 30.8 SST MEN1 GAST
10 multiple endocrine neoplasia, type i 30.8 SST SCT PDX1 MEN1 INS GCG
11 hypoglycemic coma 30.7 INS GCG
12 gastrinoma 30.7 SST SCT MEN1 INS GAST
13 hyperparathyroidism 30.6 SCT MEN1 GAST
14 carcinoid syndrome 30.6 SST MEN1
15 hyperproinsulinemia 30.5 SCT INS
16 peptic ulcer disease 30.4 SST SCT MEN1 GAST
17 type 1 diabetes mellitus 30.4 WFS1 SST PTPRN PDX1 INS IAPP
18 thyroid gland medullary carcinoma 30.4 SST MEN1 GAST
19 thyroid carcinoma, familial medullary 30.3 SST MEN1 GAST
20 primary hyperparathyroidism 30.3 MEN1 INS GAST
21 factitious disorder 30.3 INS GCK ABCC8
22 leptin deficiency or dysfunction 30.3 INS IAPP GLP1R GCG
23 dumping syndrome 30.2 SST SCT INS GCG GAST
24 hyperinsulinemic hypoglycemia, familial, 2 30.2 SST INS GAST ABCC8
25 duodenal ulcer 30.2 SST SCT INS GAST
26 hyperglycemia 30.2 SST SLC2A2 PDX1 INS IAPP GLP1R
27 glucose intolerance 30.1 SST INS GLP1R GCK GCG ABCC8
28 pancreatitis 30.1 SST SCT INS GCG
29 hyperpituitarism 30.1 SST MEN1 INS
30 acute pancreatitis 30.1 SST SCT INS
31 pancreatic agenesis 1 30.1 SCT PDX1
32 peripheral nervous system disease 30.1 WFS1 SST INS GCG
33 pancreatic cholera 30.1 SST SCT MEN1 GCG GAST
34 vipoma 30.0 SST SCT MEN1 GAST
35 pituitary gland disease 29.9 SST MEN1 INS GCG
36 carcinoid tumors, intestinal 29.9 SST MEN1 GAST
37 pancreatic endocrine carcinoma 29.9 SST MEN1 INS GCG GAST
38 body mass index quantitative trait locus 11 29.8 SLC2A2 INS IAPP GLP1R GCK GCG
39 constipation 29.8 SST INS GCG GAST
40 somatostatinoma 29.7 SST MEN1 INS GCG GAST
41 diabetes mellitus, ketosis-prone 29.7 PTPRN INSM2 INS GLP1R GCG ABCC8
42 type 2 diabetes mellitus 29.6 WFS1 SST SLC2A2 SCT PTPRN PDX1
43 maturity-onset diabetes of the young, type 3 29.6 SLC2A2 PDX1 INS GCK GCG ABCC8
44 maturity-onset diabetes of the young 29.2 WFS1 SST SLC2A2 PTPRN PDX1 INS
45 diabetes mellitus 29.2 WFS1 SST SLC2A2 SCT PTPRN2 PTPRN
46 metastatic insulinoma 11.5
47 insulinoma tumor suppressor gene locus 11.1
48 non-insulinoma pancreatogenous hypoglycemia syndrome 11.0
49 hemometra 10.5 PTPRN INSM1
50 isthmus cancer 10.5 PTPRN INSM1

Graphical network of the top 20 diseases related to Insulinoma:



Diseases related to Insulinoma

Symptoms & Phenotypes for Insulinoma

Human phenotypes related to Insulinoma:

58 31 (show all 35)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperhidrosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000975
2 tremor 58 31 hallmark (90%) Very frequent (99-80%) HP:0001337
3 recurrent hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001988
4 nonketotic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001958
5 transient global amnesia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010534
6 fasting hyperinsulinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0008283
7 palpitations 58 31 hallmark (90%) Very frequent (99-80%) HP:0001962
8 hyperinsulinemic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000825
9 abnormality of the pancreatic islet cells 58 31 hallmark (90%) Very frequent (99-80%) HP:0006476
10 seizure 31 hallmark (90%) HP:0001250
11 pituitary prolactin cell adenoma 58 31 frequent (33%) Frequent (79-30%) HP:0006767
12 generalized muscle weakness 58 31 frequent (33%) Frequent (79-30%) HP:0003324
13 polyphagia 58 31 frequent (33%) Frequent (79-30%) HP:0002591
14 increased body weight 58 31 frequent (33%) Frequent (79-30%) HP:0004324
15 primary hyperparathyroidism 58 31 frequent (33%) Frequent (79-30%) HP:0008200
16 reactive hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0012051
17 zollinger-ellison syndrome 58 31 frequent (33%) Frequent (79-30%) HP:0002044
18 fluctuations in consciousness 58 31 frequent (33%) Frequent (79-30%) HP:0007159
19 fatigue 58 31 occasional (7.5%) Occasional (29-5%) HP:0012378
20 hearing abnormality 58 31 occasional (7.5%) Occasional (29-5%) HP:0000364
21 anxiety 58 31 occasional (7.5%) Occasional (29-5%) HP:0000739
22 abnormality of vision 58 31 occasional (7.5%) Occasional (29-5%) HP:0000504
23 paresthesia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003401
24 insomnia 58 31 occasional (7.5%) Occasional (29-5%) HP:0100785
25 lethargy 58 31 occasional (7.5%) Occasional (29-5%) HP:0001254
26 coma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001259
27 abnormal rapid eye movement sleep 58 31 occasional (7.5%) Occasional (29-5%) HP:0002494
28 neuroendocrine neoplasm 58 31 occasional (7.5%) Occasional (29-5%) HP:0100634
29 neoplasm of the adrenal gland 58 31 occasional (7.5%) Occasional (29-5%) HP:0100631
30 abnormality of pain sensation 58 31 occasional (7.5%) Occasional (29-5%) HP:0010832
31 seizures 58 Very frequent (99-80%)
32 behavioral abnormality 58 Frequent (79-30%)
33 reduced consciousness/confusion 58 Frequent (79-30%)
34 hyperinsulinemia 58 Very frequent (99-80%)
35 abnormality of higher mental function 58 Occasional (29-5%)

MGI Mouse Phenotypes related to Insulinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.7 GAST INS INSM1 MEN1 PDX1 SST
2 endocrine/exocrine gland MP:0005379 9.7 ABCC8 GAST GCK GLP1R INS INSM1
3 homeostasis/metabolism MP:0005376 9.5 ABCC8 GAST GCK GLP1R IAPP INS

Drugs & Therapeutics for Insulinoma

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

(show top 50) (show all 117)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sunitinib Approved, Investigational Phase 4 557795-19-4, 341031-54-7 5329102
2 Protein Kinase Inhibitors Phase 4
3
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
4
lanreotide Approved Phase 3 108736-35-2
5
Somatostatin Approved, Investigational Phase 3 38916-34-6, 51110-01-1 53481605
6
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
7
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2 149436
8
leucovorin Approved Phase 2, Phase 3 58-05-9 6006
9
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 5310940 9887054 43805 6857599
10
Bevacizumab Approved, Investigational Phase 2, Phase 3 216974-75-3
11
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
12 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
13 Cola Phase 3
14 Anesthetics Phase 3
15 Narcotics Phase 3
16 Analgesics, Opioid Phase 3
17 Analgesics Phase 3
18 Anesthetics, Intravenous Phase 3
19 Anesthetics, General Phase 3
20 Angiopeptin Phase 3
21 Vitamin B9 Phase 2, Phase 3
22 Nutrients Phase 2, Phase 3
23 Micronutrients Phase 2, Phase 3
24 Trace Elements Phase 2, Phase 3
25 Protective Agents Phase 2, Phase 3
26 Vitamin B Complex Phase 2, Phase 3
27 Vitamins Phase 2, Phase 3
28 Folate Phase 2, Phase 3
29 Antidotes Phase 2, Phase 3
30 Antineoplastic Agents, Immunological Phase 2, Phase 3
31
Tegafur Approved, Investigational Phase 2 17902-23-7 5386
32
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
33
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
34
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
35
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
36
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
37
Melphalan Approved Phase 2 148-82-3 4053 460612
38
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166 2942
39
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
40
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
41
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
42
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
43
Capecitabine Approved, Investigational Phase 1, Phase 2 154361-50-9 60953
44
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
45
Exenatide Approved, Investigational Phase 1, Phase 2 141758-74-9 15991534
46
Pancrelipase Approved, Investigational Phase 2 53608-75-6
47
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
48
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
49
Gemcitabine Approved Phase 2 95058-81-4 60750
50
Everolimus Approved Phase 2 159351-69-6 6442177 70789204

Interventional clinical trials:

(show top 50) (show all 98)
# 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 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
5 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 Completed NCT02288377 Phase 2, Phase 3 lanreotide;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 Randomized, Double-Blinded Phase III Study of Cabozantinib Versus Placebo in Patients With Advanced Neuroendocrine Tumors After Progression on Prior Therapy (CABINET) Recruiting NCT03375320 Phase 3 Cabozantinib S-malate
8 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
9 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Terminated NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
10 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
11 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.
12 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
13 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
14 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
15 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
16 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
17 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
18 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
19 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
20 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
21 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
22 A Phase II Study Of Sunitinib In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Neuroendocrine Tumors Completed NCT01121562 Phase 2 Sunitinib
23 Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization for Metastatic Gastrointestinal Neuroendocrine Tumors Completed NCT00434109 Phase 2 Sunitinib malate
24 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
25 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
26 An Angiogenic Study in Patients With Well/Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors Treated With Everolimus Completed NCT02305810 Phase 2 Everolimus 10 mg daily
27 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. Completed NCT02713763 Phase 2 Sunitinib
28 Phase I/II Study of RAD001 in Combination With Temozolomide in Patients With Advanced Pancreatic Neuroendocrine Tumors Completed NCT00576680 Phase 1, Phase 2 RAD001;Temozolomide
29 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)
30 A Phase II Study of LEE011 (Ribociclib) in Patients With Advanced Neuroendocrine Tumors of Foregut Origin Completed NCT02420691 Phase 2 Ribociclib
31 A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors Completed NCT01024387 Phase 2 AMG 479
32 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
33 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Completed NCT02259725 Phase 2 regorafenib
34 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
35 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
36 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
37 Evaluation of the Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors Recruiting NCT04074135 Phase 2 68-Gallium DOTATATE
38 Randomized Phase II Study of Everolimus Alone Versus Everolimus Plus Bevacizumab in Patients With Locally Advanced or Metastatic Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01229943 Phase 2 Everolimus;Octreotide Acetate
39 Peptide Receptor Radionuclide Therapy With 177Lu-Dotatate Associated With Metronomic Capecitabine In Patients Affected By Aggressive Gastro-Etero-Pancreatic Neuroendocrine Tumors Active, not recruiting NCT02736500 Phase 1, Phase 2 28 GBq 177Lu-DOTATATE;22 GBq 177Lu-DOTATATE
40 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. Active, not recruiting NCT02736448 Phase 2 Capecitabine;Lu-PRRT;SS-LAR
41 A Phase II Study of MLN0128 (TAK-228) in Rapalog-Resistant Advanced Pancreatic Neuroendocrine Tumors (PNET) Active, not recruiting NCT02893930 Phase 2 Sapanisertib
42 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
43 A Phase 2 Study of Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 Capecitabine;Temozolomide
44 68Ga-NODAGA-exendin-4 PET/CT in Patients With AHH - a Prospective Comparative Evaluation of Preoperative Imaging Enrolling by invitation NCT03189953 Phase 1, Phase 2
45 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
46 Phase II Study of Panobinostat (LBH589) Given in Combination With Bortezomib (Velcade) in Patients With Pancreatic Cancer Progressing on Gemcitabine Therapy Alone or Gemcitabine in Combination Terminated NCT01056601 Phase 2 Bortezomib;Panobinostat
47 A Randomized, Open-label Phase II Multicenter Study Evaluating the Efficacy of Oral Everolimus Alone or in Combination With Pasireotide LAR i.m. in Advanced Progressive Pancreatic Neuroendocrine Tumors (PNET) - The COOPERATE-2 Study Terminated NCT01374451 Phase 2 Everolimus;Pasireotide LAR
48 Randomized Phase II Study of BEZ235 or Everolimus in Advanced Pancreatic Neuroendocrine Tumors Terminated NCT01628913 Phase 2 BEZ235;Everolimus
49 Phase II Study of Depsipeptide in Metastatic Neuroendocrine Tumors Terminated NCT00084461 Phase 2 romidepsin
50 Phase II Evaluation of Real-Time, Pharmacokinetically Guided Everolimus in Patients With Hormone Receptor Positive Breast Cancer, Pancreatic Neuroendocrine Tumors (PNET), and Renal Cell Carcinoma Terminated NCT02273752 Phase 2 Everolimus

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:

40
Pancreas, Pancreatic Islet, Pituitary, Liver, Spleen, Thyroid, T Cells
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 5952)
# 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. 54 61
20369219 2010
2
Role of JNK activation in pancreatic beta-cell death by streptozotocin. 54 61
20176078 2010
3
Expression of TAU in insulin-secreting cells and its interaction with the calcium-binding protein secretagogin. 54 61
20061514 2010
4
Ghrelin inhibits insulin secretion through the AMPK-UCP2 pathway in beta cells. 61 54
20206170 2010
5
Biological activity of EXf, a peptide analogue of exendin-4. 54 61
19919832 2010
6
Evidence for molecular mimicry between human T cell epitopes in rotavirus and pancreatic islet autoantigens. 61 54
20083660 2010
7
Pancreatic duodenal homeobox 1 protein is a novel beta-cell-specific autoantigen for type I diabetes. 61 54
19901909 2010
8
Naturally occurring glucokinase mutations are associated with defects in posttranslational S-nitrosylation. 61 54
19934346 2010
9
Glucagon-like peptide-1 receptor imaging for localization of insulinomas. 54 61
19820010 2009
10
Autophagy regulates pancreatic beta cell death in response to Pdx1 deficiency and nutrient deprivation. 54 61
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. 54 61
19567519 2009
12
Evaluation of endocrine tests. D: the prolonged fasting test for insulinoma. 61 54
19687521 2009
13
[Hyperinsulinism in infancy and childhood: when an insulin level is not always enough]. 61 54
19411227 2009
14
A new orally available glucagon-like peptide-1 receptor agonist, biotinylated exendin-4, displays improved hypoglycemic effects in db/db mice. 54 61
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. 54 61
18445139 2009
16
Weight gain in early life predicts risk of islet autoimmunity in children with a first-degree relative with type 1 diabetes. 54 61
18835948 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. 61 54
18949455 2009
18
Expression and function of IA-2 family proteins, unique neuroendocrine-specific protein-tyrosine phosphatases. 54 61
19550073 2009
19
MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells. 61 54
18775714 2008
20
Autoantibodies to GAD65 and IA-2 in canine diabetes mellitus. 61 54
18706702 2008
21
Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. 61 54
18692019 2008
22
Expression of HNF4alpha variants in pancreatic islets and Ins-1 beta cells. 61 54
18561282 2008
23
Insulinoma cell calcium-sensing receptor influences insulin secretion in a case with concurrent familial hypocalciuric hypercalcemia and malignant metastatic insulinoma. 54 61
18430790 2008
24
Differentiation of insulin secretion patterns in insulinoma. 54 61
18259806 2008
25
Mice with beta cell overexpression of glycogen synthase kinase-3beta have reduced beta cell mass and proliferation. 54 61
18219478 2008
26
Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. 54 61
18156285 2008
27
Regulation of cAMP dynamics by Ca2+ and G protein-coupled receptors in the pancreatic beta-cell: a computational approach. 61 54
17928534 2007
28
In vitro study of using calcium phosphate cement as immunoisolative device to enclose insulinoma/agarose microspheres as bioartificial pancreas. 61 54
17514757 2007
29
Expression and functional consequences of oestrogen and progesterone receptors in human insulinomas. 54 61
18045959 2007
30
Intra-operative quick insulin assay to confirm complete resection of insulinomas guided by selective arterial calcium injection (SACI). 54 61
17294212 2007
31
Rapid intraoperative insulin assay: a novel method to differentiate insulinoma from nesidioblastosis in the pediatric patient. 61 54
17958895 2007
32
[A 64-year-old female patient with recurring hypoglycaemia. Difficult aspects of diagnosis]. 54 61
17704900 2007
33
Assessment of suspected insulinoma by 48-hour fasting test: a retrospective monocentric study of 23 cases. 61 54
17611903 2007
34
[Lys40(Ahx-DTPA-111In)NH2]-Exendin-4 is a highly efficient radiotherapeutic for glucagon-like peptide-1 receptor-targeted therapy for insulinoma. 61 54
17575235 2007
35
The transcription factor PAX4 acts as a survival gene in INS-1E insulinoma cells. 61 54
17260022 2007
36
Diagnosis and minimally invasive resection of an insulinoma: report of an unusual case and review of the literature. 54 61
17521012 2007
37
Biochemistry of neuroendocrine tumours. 61 54
17382264 2007
38
Insulin responses to selective arterial calcium infusion under hyperinsulinemic euglycemic glucose clamps: case studies in adult nesidioblastosis and childhood insulinoma. 61 54
17053293 2007
39
A case of secretin-responsive insulinoma with low serum C-peptide levels. 54 61
17146148 2007
40
The PDX1 homeodomain transcription factor negatively regulates the pancreatic ductal cell-specific keratin 19 promoter. 54 61
17056599 2006
41
Trefoil factors are expressed in human and rat endocrine pancreas: differential regulation by growth hormone. 54 61
16973727 2006
42
Caveolin-1 regulates cellular trafficking and function of the glucagon-like Peptide 1 receptor. 61 54
16931572 2006
43
Two conserved domains in PCIF1 mediate interaction with pancreatic transcription factor PDX-1. 61 54
17126328 2006
44
Effects of fluoroquinolones on HERG channels and on pancreatic beta-cell ATP-sensitive K+ channels. 54 61
17027138 2006
45
Spontaneous hypoglycaemia in the presence of both anti-insulin antibody and anti-insulin receptor antibody. 61 54
17296513 2006
46
MicroRNA expression abnormalities in pancreatic endocrine and acinar tumors are associated with distinctive pathologic features and clinical behavior. 47
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). 54 61
17054445 2006
48
Sp1/Sp3 binding is associated with cell-specific expression of the glucose-dependent insulinotropic polypeptide receptor gene. 54 61
16403775 2006
49
Hypercalcitoninemia in a patient with a recurrent goitre and insulinoma: a case report. 61 54
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. 61 54
16440211 2006

Variations for Insulinoma

ClinVar genetic disease variations for Insulinoma:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 MEN1 NM_000244.3(MEN1):c.371_372del (p.Val124fs) Deletion Pathogenic 523339 rs1555166368 GRCh37: 11:64577210-64577211
GRCh38: 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
12.53 SLC2A2 PDX1 MEN1 INS GLP1R GCK
2 12.3 WFS1 PDX1 INS GCG
3
Show member pathways
12.16 SCT IAPP GLP1R GCG
4
Show member pathways
12.08 SLC2A2 INS GLP1R GCG ABCC8
5 11.87 SCT IAPP GLP1R GCG
6
Show member pathways
11.76 SLC2A2 PDX1 INS GCK
7 11.7 SLC2A2 GCK GCG
8
Show member pathways
11.64 SLC2A2 PDX1 INS GCK ABCC8
9
Show member pathways
11.1 SLC2A2 PDX1 INSM1 INS IAPP GCK
10 11 SLC2A2 PDX1 INS GCK ABCC8
11 10.68 SLC2A2 INS
12 10.28 SCT GAST

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 10.02 SST SCT INS IAPP GLP1R GCG
2 negative regulation of cell proliferation GO:0008285 9.89 SST PDX1 MIR204 MEN1 INSM1
3 cellular protein metabolic process GO:0044267 9.81 WFS1 MEN1 INS IAPP
4 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.73 IAPP GLP1R GCG
5 glucose metabolic process GO:0006006 9.61 PDX1 INS GCK
6 negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway GO:1902236 9.57 WFS1 PDX1
7 negative regulation of gluconeogenesis GO:0045721 9.55 INS GCK
8 positive regulation of glycogen biosynthetic process GO:0045725 9.54 INS GCK
9 regulation of cell cycle process GO:0010564 9.52 INSM2 INSM1
10 insulin secretion involved in cellular response to glucose stimulus GO:0035773 9.46 PTPRN2 PTPRN
11 transdifferentiation GO:0060290 9.4 PDX1 INSM1
12 negative regulation of type B pancreatic cell apoptotic process GO:2000675 9.37 WFS1 PDX1
13 glucose homeostasis GO:0042593 9.35 WFS1 PDX1 INS GCK GCG
14 type B pancreatic cell differentiation GO:0003309 9.33 PDX1 MEN1 INSM1
15 regulation of potassium ion transport GO:0043266 9.32 KCNA5 GCK
16 detection of glucose GO:0051594 9.26 PDX1 GCK
17 regulation of insulin secretion GO:0050796 9.1 SLC2A2 KCNA5 GLP1R GCK GCG ABCC8

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

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.1 SST SCT INS IAPP GCG GAST

Sources for Insulinoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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