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MCID: INS001
MIFTS: 60

Insulinoma

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Neuronal diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Insulinoma

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MalaCards integrated aliases for Insulinoma:

Name: Insulinoma 12 74 52 58 54 43 15 17 71
Islet Cell Adenoma 12 71
Experimental Organism Islet Cell Adenoma Neoplasm 71
Insulin-Producing Tumor of Islet Cells 12
Adenoma Islet Cell 54
Islet Cell Tumor 71

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:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Gastrointestinal diseases Endocrine diseases Neuronal diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


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Summaries for Insulinoma

About this section
NIH Rare Diseases : 52 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 islet cell tumor 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 Cinacalcet and Parathyroid hormone have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, pancreatic islet and liver, and related phenotypes are hyperhidrosis and tremor

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

Wikipedia : 74 An insulinoma is a tumor of the pancreas that is derived from beta cells and secretes insulin. It is a... more...

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Related Diseases for Insulinoma

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

(show top 50) (show all 431)
# Related Disease Score Top Affiliating Genes
1 islet cell tumor 32.4 SSTR2 SST MEN1 INS IAPP GCG
2 hypoglycemia 31.8 SST SLC2A2 INS IAPP GLP1R GCK
3 hyperinsulinism 31.8 SST INS IAPP GCK GCG
4 zollinger-ellison syndrome 31.5 SST MEN1 CHGA
5 glucagonoma 31.4 SST CHGA
6 hyperinsulinemic hypoglycemia 31.3 SST PDX1 MEN1 INS GCK GCG
7 multiple endocrine neoplasia, type i 31.3 SST PDX1 MEN1 INS GCG CHGA
8 neuroendocrine tumor 31.2 SSTR2 SST MEN1 INSM1 GCG CHGA
9 adenoma 31.1 SSTR5 SSTR2 SST MEN1 CHGA
10 neuroendocrine carcinoma 31.1 SST MEN1 GCG CHGA
11 hypoglycemic coma 31.0 INS GCG
12 peptic ulcer disease 31.0 SST MEN1 INS
13 pituitary tumors 30.9 SSTR5 SST MEN1
14 primary hyperparathyroidism 30.9 MEN1 INS CHGA
15 dumping syndrome 30.9 SST INS GCG
16 gastrinoma 30.8 SSTR5 SSTR2 SST MEN1 INS CHGA
17 vipoma 30.7 SST MEN1 CHGA
18 glucose intolerance 30.5 INS IAPP GLP1R GCK GCG
19 hyperglycemia 30.5 SST SLC2A2 PDX1 INS IAPP GLP1R
20 urinary bladder small cell neuroendocrine carcinoma 30.4 INSM1 CHGA
21 pancreatitis 30.3 SST PDX1 INS GCG
22 paraganglioma 30.3 SST MEN1 CHGA
23 thyroid gland medullary carcinoma 30.3 SSTR5 SSTR2 SST MEN1 CHGA
24 thyroid carcinoma, familial medullary 30.3 SSTR5 SSTR2 SST MEN1 CHGA
25 pituitary adenoma 30.3 SSTR5 SSTR2 SST MEN1 CHGA
26 adrenal carcinoma 30.2 SST MEN1 CHGA
27 pituitary adenoma, prolactin-secreting 30.2 SSTR5 SSTR2 SST MEN1 CHGA
28 maturity-onset diabetes of the young, type 3 30.2 SLC2A2 PDX1 INS GCK
29 endocrine gland cancer 30.2 SST MIR204 IAPP GCG CHGA
30 pancreatic cholera 30.1 SST MEN1 GCG CHGA
31 acromegaly 30.1 SSTR5 SSTR2 SST MEN1 INS
32 diabetes mellitus, ketosis-prone 30.1 PTPRN INSM2 INS GLP1R GCG
33 constipation 30.1 SST INS GCG CHGA
34 carcinoid tumors, intestinal 30.0 SSTR2 SST MEN1 CHGA
35 hyperpituitarism 29.9 SSTR5 SSTR2 SST MEN1 INS
36 body mass index quantitative trait locus 11 29.9 SLC2A2 INS IAPP GLP1R GCK GCG
37 small cell cancer of the lung 29.9 SSTR2 SST CHGA
38 hypertension, essential 29.9 SST MEN1 KCNA5 INS IAPP GCG
39 pancreatic endocrine carcinoma 29.8 SSTR5 SSTR2 SST MEN1 GCG CHGA
40 diabetes mellitus, noninsulin-dependent 29.8 WFS1 SST SLC2A2 PTPRN PDX1 INS
41 diabetes mellitus, type i 29.7 WFS1 SST SLC2A2 PTPRN2 PTPRN PDX1
42 pituitary gland disease 29.7 SSTR5 SSTR2 SST MEN1 INS GCG
43 somatostatinoma 29.7 SSTR2 SST MEN1 INS GCG CHGA
44 diabetes mellitus 29.6 WFS1 SST SLC2A2 PTPRN2 PTPRN PDX1
45 maturity-onset diabetes of the young 29.4 WFS1 SST SLC2A2 PTPRN PDX1 INS
46 metastatic insulinoma 12.7
47 insulinoma tumor suppressor gene locus 12.4
48 non-insulinoma pancreatogenous hypoglycemia syndrome 12.3
49 insulinomatosis and diabetes mellitus 11.6
50 neuroblastoma 10.8

Graphical network of the top 20 diseases related to Insulinoma:



Diseases related to Insulinoma
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Symptoms & Phenotypes for Insulinoma

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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 hyperinsulinemic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000825
5 nonketotic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001958
6 transient global amnesia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010534
7 fasting hyperinsulinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0008283
8 palpitations 58 31 hallmark (90%) Very frequent (99-80%) HP:0001962
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 generalized muscle weakness 58 31 frequent (33%) Frequent (79-30%) HP:0003324
12 pituitary prolactin cell adenoma 58 31 frequent (33%) Frequent (79-30%) HP:0006767
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 neoplasm of the adrenal gland 58 31 occasional (7.5%) Occasional (29-5%) HP:0100631
29 neuroendocrine neoplasm 58 31 occasional (7.5%) Occasional (29-5%) HP:0100634
30 abnormality of pain sensation 58 31 occasional (7.5%) Occasional (29-5%) HP:0010832
31 behavioral abnormality 58 Frequent (79-30%)
32 seizures 58 Very frequent (99-80%)
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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.9 CHGA GCK GLP1R INS INSM1 MEN1
2 digestive/alimentary MP:0005381 9.8 INS INSM1 MEN1 PDX1 SST SSTR2
3 growth/size/body region MP:0005378 9.73 CHGA GCK GLP1R IAPP INS INSM1
4 homeostasis/metabolism MP:0005376 9.53 CHGA GCK GLP1R IAPP INS INSM1
Sources

Drugs & Therapeutics for Insulinoma

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

(show top 50) (show all 126)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cinacalcet Approved Phase 3 226256-56-0 156419
2
Parathyroid hormone Approved, Investigational Phase 3 9002-64-6
3
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
4
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
5
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2
6
leucovorin Approved Phase 2, Phase 3 58-05-9 6006 143
7
Sunitinib Approved, Investigational Phase 3 557795-19-4, 341031-54-7 5329102
8
Bevacizumab Approved, Investigational Phase 2, Phase 3 216974-75-3
9
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 43805 6857599 5310940 9887054
10
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
11 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
12 Hormones Phase 3
13 Hormone Antagonists Phase 3
14 Liver Extracts Phase 3
15 Calcium, Dietary Phase 3
16 Cola Phase 3
17 Anesthetics Phase 3
18 Narcotics Phase 3
19 Analgesics Phase 3
20 Analgesics, Opioid Phase 3
21 Anesthetics, General Phase 3
22 Anesthetics, Intravenous Phase 3
23 Antimetabolites Phase 2, Phase 3
24 Angiogenesis Inhibitors Phase 3
25 Micronutrients Phase 2, Phase 3
26 Vitamins Phase 2, Phase 3
27 Antidotes Phase 2, Phase 3
28 Vitamin B Complex Phase 2, Phase 3
29 Folate Phase 2, Phase 3
30 Nutrients Phase 2, Phase 3
31 Trace Elements Phase 2, Phase 3
32 Vitamin B9 Phase 2, Phase 3
33 Protective Agents Phase 2, Phase 3
34 Antineoplastic Agents, Immunological Phase 2, Phase 3
35
Calcium Nutraceutical Phase 3 7440-70-2 271
36
Tegafur Approved, Investigational Phase 2 17902-23-7 5386
37
Glycerol Approved, Investigational Phase 2 56-81-5 753
38
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
39
Cisplatin Approved Phase 2 15663-27-1 2767 441203 84093
40
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
41
Melphalan Approved Phase 2 148-82-3 460612 4053
42
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
43
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
44
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
45
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
46
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
47
Temozolomide Approved, Investigational Phase 1, Phase 2 85622-93-1 5394
48
lanreotide Approved Phase 2 108736-35-2
49
Exenatide Approved, Investigational Phase 1, Phase 2 141758-74-9 15991534
50
Pancrelipase Approved, Investigational Phase 2 53608-75-6

Interventional clinical trials:

(show top 50) (show all 105)
# 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 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)
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 Cinacalcet Actions in Familial Primary Hyperparathyroidism Completed NCT00325104 Phase 3
7 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
8 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
9 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
10 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
11 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
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 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.
14 A Randomised, Double-blind, Placebo-controlled Trial of Intranasal Insulin (440 IU) in Children and Young Adults at Risk of Type 1 Diabetes: Intranasal Insulin Trial II Unknown status NCT00336674 Phase 2
15 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
16 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
17 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
18 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
19 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
20 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
21 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
22 A Phase II Study Of Sunitinib In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Neuroendocrine Tumors Completed NCT01121562 Phase 2 Sunitinib
23 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
24 Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization for Metastatic Gastrointestinal Neuroendocrine Tumors Completed NCT00434109 Phase 2 Sunitinib malate
25 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)
26 A Phase II Study of LEE011 (Ribociclib) in Patients With Advanced Neuroendocrine Tumors of Foregut Origin Completed NCT02420691 Phase 2 Ribociclib
27 A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors Completed NCT01024387 Phase 2 AMG 479
28 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
29 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
30 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
31 An Angiogenic Study in Patients With Well/Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors Treated With Everolimus Completed NCT02305810 Phase 2 Everolimus 10 mg daily
32 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
33 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
34 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
35 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
36 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
37 An Exploratory, Open Label Study of Anti-inflammatory Therapy With Anakinra in Children With Newly Diagnosed Type 1 Diabetes Mellitus Completed NCT00645840 Phase 1, Phase 2 Anakinra
38 Evaluation of the Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors Recruiting NCT04074135 Phase 2 68-Gallium DOTATATE
39 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
40 A Phase II Study of MLN0128 (TAK-228) in Rapalog-Resistant Advanced Pancreatic Neuroendocrine Tumors (PNET) Active, not recruiting NCT02893930 Phase 2 Sapanisertib
41 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
42 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
43 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
44 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Active, not recruiting NCT02259725 Phase 2 regorafenib
45 A Phase 2 Study of Capecitabine, Temozolomide and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 capecitabine;temozolomide
46 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
47 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
48 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
49 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
50 Randomized Phase II Study of BEZ235 or Everolimus in Advanced Pancreatic Neuroendocrine Tumors Terminated NCT01628913 Phase 2 BEZ235;Everolimus

Search NIH Clinical Center for Insulinoma

Cochrane evidence based reviews: insulinoma

Sources

Genetic Tests for Insulinoma

About this section
Sources

Anatomical Context for Insulinoma

About this section

MalaCards organs/tissues related to Insulinoma:

40
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
Sources

Publications for Insulinoma

About this section

Articles related to Insulinoma:

(show top 50) (show all 5767)
# 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. 61 54
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. 61 54
19919832 2010
6
Evidence for molecular mimicry between human T cell epitopes in rotavirus and pancreatic islet autoantigens. 54 61
20083660 2010
7
Naturally occurring glucokinase mutations are associated with defects in posttranslational S-nitrosylation. 61 54
19934346 2010
8
Pancreatic duodenal homeobox 1 protein is a novel beta-cell-specific autoantigen for type I diabetes. 54 61
19901909 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. 54 61
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. 61 54
18445139 2009
16
Expression and function of IA-2 family proteins, unique neuroendocrine-specific protein-tyrosine phosphatases. 61 54
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. 61 54
18949455 2009
18
Weight gain in early life predicts risk of islet autoimmunity in children with a first-degree relative with type 1 diabetes. 61 54
18835948 2009
19
Autoantibodies to GAD65 and IA-2 in canine diabetes mellitus. 54 61
18706702 2008
20
MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells. 54 61
18775714 2008
21
Expression of HNF4alpha variants in pancreatic islets and Ins-1 beta cells. 54 61
18561282 2008
22
Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. 54 61
18692019 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. 61 54
18219478 2008
26
Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. 61 54
18156285 2008
27
Regulation of cAMP dynamics by Ca2+ and G protein-coupled receptors in the pancreatic beta-cell: a computational approach. 54 61
17928534 2007
28
Expression and functional consequences of oestrogen and progesterone receptors in human insulinomas. 61 54
18045959 2007
29
In vitro study of using calcium phosphate cement as immunoisolative device to enclose insulinoma/agarose microspheres as bioartificial pancreas. 54 61
17514757 2007
30
Intra-operative quick insulin assay to confirm complete resection of insulinomas guided by selective arterial calcium injection (SACI). 61 54
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. 54 61
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. 61 54
17521012 2007
37
Biochemistry of neuroendocrine tumours. 54 61
17382264 2007
38
Insulin responses to selective arterial calcium infusion under hyperinsulinemic euglycemic glucose clamps: case studies in adult nesidioblastosis and childhood insulinoma. 54 61
17053293 2007
39
A case of secretin-responsive insulinoma with low serum C-peptide levels. 54 61
17146148 2007
40
Trefoil factors are expressed in human and rat endocrine pancreas: differential regulation by growth hormone. 54 61
16973727 2006
41
Two conserved domains in PCIF1 mediate interaction with pancreatic transcription factor PDX-1. 61 54
17126328 2006
42
Spontaneous hypoglycaemia in the presence of both anti-insulin antibody and anti-insulin receptor antibody. 54 61
17296513 2006
43
Caveolin-1 regulates cellular trafficking and function of the glucagon-like Peptide 1 receptor. 54 61
16931572 2006
44
Effects of fluoroquinolones on HERG channels and on pancreatic beta-cell ATP-sensitive K+ channels. 61 54
17027138 2006
45
The PDX1 homeodomain transcription factor negatively regulates the pancreatic ductal cell-specific keratin 19 promoter. 54 61
17056599 2006
46
MicroRNA expression abnormalities in pancreatic endocrine and acinar tumors are associated with distinctive pathologic features and clinical behavior. 46
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
Sources

Variations for Insulinoma

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ClinVar genetic disease variations for Insulinoma:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 MEN1 NM_000244.3(MEN1):c.371_372del (p.Val124fs)deletion Pathogenic 523339 rs1555166368 11:64577210-64577211 11:64809738-64809739
Sources

Expression for Insulinoma

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Search GEO for disease gene expression data for Insulinoma.
Sources

Pathways for Insulinoma

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Pathways related to Insulinoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Aldosterone synthesis and secretion 36
Show member pathways
 12.47 SLC2A2 PDX1 MEN1 INS GLP1R GCK
2
Glucose / Energy Metabolism 4
12.27 WFS1 PDX1 INS GCG
3
Integration of energy metabolism 65
Show member pathways
 12.07 SLC2A2 INS GLP1R GCG
4
cAMP signaling pathway 36
11.99 SSTR5 SSTR2 SST GLP1R GCG
5
Hepatic ABC Transporters 63
Show member pathways
 11.76 SLC2A2 PDX1 INS GCK
6
Glucagon signaling pathway 36
11.66 SLC2A2 GCK GCG
7
Type II diabetes mellitus 36 1
Show member pathways
 11.6 SLC2A2 PDX1 INS GCK
8
Regulation of beta-cell development 65
Show member pathways
 11.1 SLC2A2 PDX1 INSM1 INS IAPP GCK
9
FOXA2 and FOXA3 transcription factor networks 1
10.9 SLC2A2 PDX1 INS GCK
10
Cori Cycle 1
10.64 SLC2A2 INS
Sources

GO Terms for Insulinoma

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

# Name GO ID Score Top Affiliating Genes
1 cell GO:0005623 9.5 PTPRN2 PTPRN MEN1 KCNA5 IAPP GLP1R
2 secretory granule GO:0030141 9.33 PTPRN2 PTPRN CHGA
3 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 19)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 10.02 SSTR5 SSTR2 SST INS IAPP GLP1R
2 cellular protein metabolic process GO:0044267 9.81 WFS1 MEN1 INS IAPP
3 negative regulation of cell proliferation GO:0008285 9.8 SSTR5 SSTR2 SST PDX1 MIR204 MEN1
4 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.75 IAPP GLP1R GCG
5 glucose metabolic process GO:0006006 9.63 PDX1 INS GCK
6 negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway GO:1902236 9.58 WFS1 PDX1
7 negative regulation of gluconeogenesis GO:0045721 9.57 INS GCK
8 positive regulation of glycogen biosynthetic process GO:0045725 9.56 INS GCK
9 regulation of potassium ion transport GO:0043266 9.55 KCNA5 GCK
10 glucose homeostasis GO:0042593 9.55 WFS1 SSTR5 PDX1 INS GCK
11 regulation of cell cycle process GO:0010564 9.54 INSM2 INSM1
12 negative regulation of blood vessel diameter GO:0097756 9.52 INS CHGA
13 insulin secretion involved in cellular response to glucose stimulus GO:0035773 9.49 PTPRN2 PTPRN
14 transdifferentiation GO:0060290 9.43 PDX1 INSM1
15 negative regulation of type B pancreatic cell apoptotic process GO:2000675 9.4 WFS1 PDX1
16 somatostatin signaling pathway GO:0038170 9.37 SSTR5 SSTR2
17 detection of glucose GO:0051594 9.32 PDX1 GCK
18 type B pancreatic cell differentiation GO:0003309 9.13 PDX1 MEN1 INSM1
19 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 somatostatin receptor activity GO:0004994 8.96 SSTR5 SSTR2
2 hormone activity GO:0005179 8.92 SST INS IAPP GCG
Sources

Sources for Insulinoma

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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