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 74 52 58 54 6 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:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


Summaries for Insulinoma

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 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 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 seizures and hyperhidrosis

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...

Related Diseases for Insulinoma

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

(show top 50) (show all 433)
# Related Disease Score Top Affiliating Genes
1 islet cell tumor 32.7 SST MEN1 INS IAPP GCG CHGA
2 hyperinsulinism 31.8 SST INS IAPP GCK GCG
3 hypoglycemia 31.8 SST SLC2A2 INS IAPP GLP1R GCK
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 adenoma 31.2 SSTR5 SSTR2 SST MEN1 CHGA
9 neuroendocrine tumor 31.1 SSTR2 SST MEN1 INSM1 GCG CHGA
10 neuroendocrine carcinoma 31.1 SST MEN1 GCG CHGA
11 hypoglycemic coma 31.1 INS GCG
12 pituitary tumors 31.0 SSTR5 SST MEN1
13 hirata disease 30.9 INS GCG
14 dumping syndrome 30.9 SST INS GCG
15 primary hyperparathyroidism 30.8 MEN1 INS CHGA
16 gastrinoma 30.8 SSTR5 SSTR2 SST MEN1 INS CHGA
17 vipoma 30.8 SST MEN1 CHGA
18 glucose intolerance 30.5 INS IAPP GLP1R GCK GCG
19 hyperglycemia 30.5 SST SLC2A2 PDX1 INS IAPP GLP1R
20 merkel cell carcinoma 30.4 SSTR2 SST CHGA
21 paraganglioma 30.4 SST MEN1 CHGA
22 diabetes mellitus, ketosis-prone 30.3 PTPRN INSM2 INS GCG
23 pituitary adenoma 30.2 SSTR5 SSTR2 SST MEN1 CHGA
24 adrenal carcinoma 30.2 SST MEN1 CHGA
25 pancreatic cholera 30.2 SST MEN1 GCG CHGA
26 pituitary adenoma, prolactin-secreting 30.1 SSTR5 SSTR2 SST MEN1 CHGA
27 maturity-onset diabetes of the young, type 3 30.1 SLC2A2 PDX1 INS GCK
28 acromegaly 30.1 SSTR5 SSTR2 SST MEN1 INS
29 thyroid gland medullary carcinoma 30.0 SSTR5 SSTR2 SST MEN1 CHGA
30 carcinoid tumors, intestinal 30.0 SSTR2 SST MEN1 CHGA
31 endocrine gland cancer 30.0 SST MIR204 IAPP GCG CHGA
32 thyroid carcinoma, familial medullary 30.0 SSTR5 SSTR2 SST MEN1 CHGA
33 polycystic liver disease 30.0 SSTR5 SSTR2 SST
34 hyperpituitarism 29.9 SSTR5 SSTR2 SST MEN1 INS
35 body mass index quantitative trait locus 11 29.9 SLC2A2 INS IAPP GLP1R GCK GCG
36 hypertension, essential 29.8 SST MEN1 KCNA5 INS IAPP GCG
37 diabetes mellitus, noninsulin-dependent 29.7 WFS1 SST SLC2A2 PTPRN PDX1 INS
38 pituitary gland disease 29.7 SSTR5 SSTR2 SST MEN1 INS GCG
39 somatostatinoma 29.7 SSTR2 SST MEN1 INS GCG CHGA
40 pancreatic endocrine carcinoma 29.7 SSTR5 SSTR2 SST MEN1 INS GCG
41 diabetes mellitus, insulin-dependent 29.7 WFS1 SST SLC2A2 PTPRN2 PTPRN PDX1
42 diabetes mellitus 29.5 WFS1 SST SLC2A2 PTPRN2 PTPRN PDX1
43 maturity-onset diabetes of the young 29.3 WFS1 SST SLC2A2 PTPRN PDX1 INS
44 metastatic insulinoma 12.7
45 insulinoma tumor suppressor gene locus 12.4
46 adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia 12.3
47 insulinomatosis and diabetes mellitus 11.6
48 neuroblastoma 10.8
49 multiple endocrine neoplasia 10.8
50 isthmus cancer 10.6 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 34)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 seizures 58 31 hallmark (90%) Very frequent (99-80%) HP:0001250
2 hyperhidrosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000975
3 tremor 58 31 hallmark (90%) Very frequent (99-80%) HP:0001337
4 hyperinsulinemic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000825
5 fasting hyperinsulinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0008283
6 recurrent hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001988
7 palpitations 58 31 hallmark (90%) Very frequent (99-80%) HP:0001962
8 transient global amnesia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010534
9 nonketotic hypoglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001958
10 abnormality of the pancreatic islet cells 58 31 hallmark (90%) Very frequent (99-80%) HP:0006476
11 generalized muscle weakness 58 31 frequent (33%) Frequent (79-30%) HP:0003324
12 zollinger-ellison syndrome 58 31 frequent (33%) Frequent (79-30%) HP:0002044
13 pituitary prolactin cell adenoma 58 31 frequent (33%) Frequent (79-30%) HP:0006767
14 polyphagia 58 31 frequent (33%) Frequent (79-30%) HP:0002591
15 increased body weight 58 31 frequent (33%) Frequent (79-30%) HP:0004324
16 reactive hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0012051
17 fluctuations in consciousness 58 31 frequent (33%) Frequent (79-30%) HP:0007159
18 primary hyperparathyroidism 58 31 frequent (33%) Frequent (79-30%) HP:0008200
19 fatigue 58 31 occasional (7.5%) Occasional (29-5%) HP:0012378
20 abnormality of vision 58 31 occasional (7.5%) Occasional (29-5%) HP:0000504
21 hearing abnormality 58 31 occasional (7.5%) Occasional (29-5%) HP:0000364
22 anxiety 58 31 occasional (7.5%) Occasional (29-5%) HP:0000739
23 paresthesia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003401
24 neuroendocrine neoplasm 58 31 occasional (7.5%) Occasional (29-5%) HP:0100634
25 coma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001259
26 abnormal rapid eye movement sleep 58 31 occasional (7.5%) Occasional (29-5%) HP:0002494
27 insomnia 58 31 occasional (7.5%) Occasional (29-5%) HP:0100785
28 lethargy 58 31 occasional (7.5%) Occasional (29-5%) HP:0001254
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 behavioral abnormality 58 Frequent (79-30%)
32 reduced consciousness/confusion 58 Frequent (79-30%)
33 hyperinsulinemia 58 Very frequent (99-80%)
34 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

Drugs & Therapeutics for Insulinoma

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

(show top 50) (show all 132)
# 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
lanreotide Approved Phase 2, Phase 3 108736-35-2
5
Sunitinib Approved, Investigational Phase 3 341031-54-7, 557795-19-4 5329102
6
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
7 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
8 Hormone Antagonists Phase 3
9 Hormones Phase 3
10 Liver Extracts Phase 3
11 Calcium, Dietary Phase 3
12 Cola Phase 3
13 Central Nervous System Depressants Phase 3
14 Calcimimetic Agents Phase 3
15 Narcotics Phase 3
16 Anesthetics Phase 3
17 Anesthetics, General Phase 3
18 Analgesics, Opioid Phase 3
19 Adjuvants, Anesthesia Phase 3
20 Anesthetics, Intravenous Phase 3
21 Analgesics Phase 3
22 Angiopeptin Phase 2, Phase 3
23 Angiogenesis Inhibitors Phase 3
24
Tegafur Approved, Investigational Phase 2 17902-23-7 5386
25
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
26
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
27
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
28
Nicotinamide Approved, Investigational Phase 2 98-92-0 936
29
Melphalan Approved Phase 2 148-82-3 4053 460612
30
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
31
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
32
Capecitabine Approved, Investigational Phase 1, Phase 2 154361-50-9 60953
33
Temozolomide Approved, Investigational Phase 1, Phase 2 85622-93-1 5394
34
Bevacizumab Approved, Investigational Phase 2 216974-75-3
35
Zinc Approved, Investigational Phase 2 7440-66-6 32051
36
Glycerol Approved, Investigational Phase 2 56-81-5 753
37
Exenatide Approved, Investigational Phase 1, Phase 2 141758-74-9 15991534
38
Pancrelipase Approved, Investigational Phase 2 53608-75-6
39
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
40
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
41
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
42
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
43
Gemcitabine Approved Phase 2 95058-81-4 60750
44
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
45
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
46
Vitamin A Approved, Nutraceutical, Vet_approved Phase 2 68-26-8, 11103-57-4, 22737-96-8 445354 9904001
47
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
48
Imetelstat Investigational Phase 2 868169-64-6
49 Dactolisib Investigational Phase 2 915019-65-7
50 interferons Phase 2

Interventional clinical trials:

(show top 50) (show all 104)
# 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 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)
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 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
9 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
10 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
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 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.
13 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
14 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
15 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
16 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
17 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
18 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
19 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
20 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
21 A Phase II Study Of Sunitinib In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Neuroendocrine Tumors Completed NCT01121562 Phase 2 Sunitinib
22 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
23 Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization for Metastatic Gastrointestinal Neuroendocrine Tumors Completed NCT00434109 Phase 2 Sunitinib malate
24 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)
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 A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors Completed NCT01024387 Phase 2 AMG 479
27 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
28 An Angiogenic Study in Patients With Well/Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors Treated With Everolimus Completed NCT02305810 Phase 2 Everolimus 10 mg daily
29 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
30 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
31 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
32 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
33 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
34 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
35 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
36 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
37 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. Active, not recruiting NCT02713763 Phase 2 Sunitinib
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 A Phase II Study of Capecitabine, Temozolomide and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 capecitabine;temozolomide
41 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Active, not recruiting NCT02259725 Phase 2 regorafenib
42 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 Active, not recruiting NCT00336674 Phase 2
43 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
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 A Phase II Study of MLN0128 (TAK-228) in Rapalog-Resistant Advanced Pancreatic Neuroendocrine Tumors (PNET) Suspended NCT02893930 Phase 2 Sapanisertib
46 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
47 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
48 Randomized Phase II Study of BEZ235 or Everolimus in Advanced Pancreatic Neuroendocrine Tumors Terminated NCT01628913 Phase 2 BEZ235;Everolimus
49 A Multicenter Phase II Open Study Coupled With a Translational Assessment of Biomarkers Predictive of Response to Sunitinib in Patients With Poorly-differentiated Advanced/Inoperable NEURO-Endocrine Tumors. Terminated NCT01215578 Phase 2 Sutent
50 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

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, Liver, Pituitary, Testes, 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 5677)
# 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
Ghrelin inhibits insulin secretion through the AMPK-UCP2 pathway in beta cells. 54 61
20206170 2010
4
Expression of TAU in insulin-secreting cells and its interaction with the calcium-binding protein secretagogin. 54 61
20061514 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. 54 61
20083660 2010
7
Naturally occurring glucokinase mutations are associated with defects in posttranslational S-nitrosylation. 54 61
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]. 54 61
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
Expression and function of IA-2 family proteins, unique neuroendocrine-specific protein-tyrosine phosphatases. 54 61
19550073 2009
17
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
18
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. 54 61
18949455 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
Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. 54 61
18692019 2008
22
Expression of HNF4alpha variants in pancreatic islets and Ins-1 beta cells. 54 61
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
In vitro study of using calcium phosphate cement as immunoisolative device to enclose insulinoma/agarose microspheres as bioartificial pancreas. 54 61
17514757 2007
28
Expression and functional consequences of oestrogen and progesterone receptors in human insulinomas. 54 61
18045959 2007
29
Regulation of cAMP dynamics by Ca2+ and G protein-coupled receptors in the pancreatic beta-cell: a computational approach. 54 61
17928534 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. 54 61
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
The transcription factor PAX4 acts as a survival gene in INS-1E insulinoma cells. 54 61
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. 54 61
17575235 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. 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
Two conserved domains in PCIF1 mediate interaction with pancreatic transcription factor PDX-1. 54 61
17126328 2006
41
Trefoil factors are expressed in human and rat endocrine pancreas: differential regulation by growth hormone. 54 61
16973727 2006
42
Spontaneous hypoglycaemia in the presence of both anti-insulin antibody and anti-insulin receptor antibody. 54 61
17296513 2006
43
Effects of fluoroquinolones on HERG channels and on pancreatic beta-cell ATP-sensitive K+ channels. 54 61
17027138 2006
44
Caveolin-1 regulates cellular trafficking and function of the glucagon-like Peptide 1 receptor. 54 61
16931572 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. 54 61
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. 54 61
16440211 2006

Variations for Insulinoma

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

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.47 SLC2A2 PDX1 MEN1 INS GLP1R GCK
2 12.27 WFS1 PDX1 INS GCG
3
Show member pathways
12.07 SLC2A2 INS GLP1R GCG
4 11.99 SSTR5 SSTR2 SST GLP1R GCG
5
Show member pathways
11.76 SLC2A2 PDX1 INS GCK
6 11.66 SLC2A2 GCK GCG
7
Show member pathways
11.6 SLC2A2 PDX1 INS GCK
8
Show member pathways
11.1 SLC2A2 PDX1 INSM1 INS IAPP GCK
9 10.9 SLC2A2 PDX1 INS GCK
10 10.64 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 cell GO:0005623 9.7 PTPRN2 PTPRN MEN1 KCNA5 IAPP GLP1R
2 secretory granule GO:0030141 9.43 PTPRN2 PTPRN CHGA
3 transport vesicle membrane GO:0030658 9.13 PTPRN2 PTPRN CHGA
4 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 10.04 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 9.73 IAPP GLP1R GCG
5 glucose metabolic process GO:0006006 9.65 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 9.58 INS GCK
8 positive regulation of glycogen biosynthetic process GO:0045725 9.57 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 transdifferentiation GO:0060290 9.46 PDX1 INSM1
14 insulin secretion involved in cellular response to glucose stimulus GO:0035773 9.43 PTPRN2 PTPRN
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 for Insulinoma

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
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