PNET
MCID: ISL001
MIFTS: 56

Islet Cell Tumor (PNET)

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

Aliases & Classifications for Islet Cell Tumor

MalaCards integrated aliases for Islet Cell Tumor:

Name: Islet Cell Tumor 12 52 54 15 17 71
Pancreatic Neuroendocrine Tumor 52 58 36 17
Neuroendocrine Tumor of Pancreas 52 58 6
Well-Differentiated Neuroendocrine Neoplasm of Pancreas 52 58
Well-Differentiated Pancreatic Neuroendocrine Neoplasm 52 58
Well-Differentiated Nen of Pancreas 52 58
Well-Differentiated Pancreatic Nen 52 58
Pancreatic Net 52 58
Pnet 52 58
Well Differentiated Pancreatic Endocrine Tumor 71
Malignant Neoplasm of Endocrine Pancreas 71
Malignant Tumor of Endocrine Pancreas 12
Malignant Pancreatic Endocrine Tumor 12
Pancreatic Neuroendocrine Neoplasm 52
Pancreatic Endocrine Carcinoma 71
Pancreatic Endocrine Neoplasm 12
Pancreatic Endocrine Tumor 52
Endocrine Pancreas Cancer 12
Islet Cell Neoplasm 12
Adenoma, Islet Cell 43
Islet Cell Tumour 12

Characteristics:

Orphanet epidemiological data:

58
neuroendocrine tumor of pancreas
Inheritance: Autosomal dominant,Not applicable; Prevalence: 1-9/1000000 (United States),1-9/100000 (Japan),1-9/1000000 (France),1-9/1000000 (Europe),1-5/10000 (United States); Age of onset: Adult; Age of death: adult,elderly;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:1799
KEGG 36 H00045
MeSH 43 D007516
NCIt 49 C27031
SNOMED-CT 67 76345009
ICD10 32 C25.4 D13.7
ICD10 via Orphanet 33 E16.8
UMLS via Orphanet 72 C0242363
Orphanet 58 ORPHA97253
UMLS 71 C0242363 C0496784 C1328479 more

Summaries for Islet Cell Tumor

NIH Rare Diseases : 52 A pancreatic neuroendocrine tumor , also called an islet cell tumor, is a type of neuroendocrine tumor (NET) that typically arises in the pancreas . However in some cases, a pancreatic NET occurs outside of the pancreas. A NET arises from cells that produce hormones , so the tumor can also produce hormones. It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs usually grow slowly over many years, but there are fast-growing forms. Pancreatic NETs are called either functional or nonfunctional. A functional pancreatic NET causes specific symptoms because it makes extra hormones, such as gastrin, insulin, or glucagon. Examples of types of functional pancreatic NETs include insulinomas , glucagonomas , gastrinomas , VIPomas , and somatostatinomas . Symptoms depend on the type of hormone being made. A nonfunctional pancreatic NET generally does not cause specific symptoms, but may eventually cause symptoms relating to its location and size as it grows or spreads. Pancreatic NETs can be hard to diagnosis, often not identified until 5 to 10 years after they begin to grow. Most pancreatic NETs are not inherited and occur sporadically in people with no family history of NETs. However, about 10% are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (MEN1), which has autosomal dominant inheritance. Treatment options for each person depend on many factors including the type of tumor, its location, whether it has spread to other parts of the body (metastasized), and symptoms present. Treatment options may include surgery to remove all or part of the tumor (and sometimes the lymph nodes ), and/or nonsurgical treatments to shrink the tumor, stop it from growing, or alleviate symptoms. The chance of recovery (prognosis ) also depends upon these factors and differs from person to person.

MalaCards based summary : Islet Cell Tumor, also known as pancreatic neuroendocrine tumor, is related to non-functioning pancreatic endocrine tumor and ewing sarcoma. An important gene associated with Islet Cell Tumor is BRCA2 (BRCA2 DNA Repair Associated), and among its related pathways/superpathways are Signaling by GPCR and Peptide ligand-binding receptors. The drugs Doxorubicin and Oxaliplatin have been mentioned in the context of this disorder. Affiliated tissues include pancreatic islet cells, pancreas and brain, and related phenotypes are homeostasis/metabolism and behavior/neurological

Disease Ontology : 12 A pancreatic cancer that is located in the pancreatic islet cells.

KEGG : 36 Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. They may occur sporadically or in association with a genetic syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Landau (VHL) syndrome, neurofibromatosis type 1, or tuberous sclerosis. The most frequent genetic alterations in PNET occur in MEN1 (Multiple Endocrine Neoplasia-1 Gene), DAXX/ATRX (Death-Domain Associated Protein/Mental Retardation Syndrome X-Linked Genes) and the mTOR pathway (Mammalian Target of Rapamycin). A germline mutation in the MEN1 tumor suppressor gene causes MEN1, the above-mentioned autosomal dominant hereditary syndrome. Mutations of DAXX and ATRX are common and related to altered telomeres but not to prognosis.

Related Diseases for Islet Cell Tumor

Diseases related to Islet Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 633)
# Related Disease Score Top Affiliating Genes
1 non-functioning pancreatic endocrine tumor 34.6 SST MEN1 GAST CHGA
2 ewing sarcoma 33.2 SYP RET IGF2 IGF1 CHGA
3 insulinoma 32.7 SST MEN1 INS IGF2 IAPP GAST
4 pancreatic endocrine carcinoma 32.5 SYP SST MEN1 INS GAST CHGA
5 vipoma 32.5 VIP SST MEN1 GHRH GH1 GAST
6 somatostatinoma 32.4 VIP SST MEN1 INS GHRH GAST
7 glucagonoma 31.4 VIP SST GAST CHGA
8 zollinger-ellison syndrome 31.3 SST MEN1 GHRH GAST CHGA
9 neuroendocrine tumor 31.3 VIP SYP SST RET MEN1 IGF1
10 multiple endocrine neoplasia 31.3 RET MEN1 CALCA
11 peptic ulcer disease 31.0 SST MEN1 GAST
12 hyperinsulinism 31.0 SST INS IGF2 IGF1 IAPP GH1
13 hypoglycemia 31.0 SST INS IGF2 IGF1 IAPP GHRH
14 fasting hypoglycemia 30.9 IGF2 IGF1
15 autoimmune atrophic gastritis 30.9 GAST CHGA
16 hyperinsulinemic hypoglycemia 30.8 SST MEN1 INS IGF2
17 teratoma 30.8 SYP SST INS CHGA
18 pancreatic somatostatinoma 30.7 SST CALCA
19 non-functioning pituitary adenoma 30.7 SST GH1
20 gastrinoma 30.7 VIP SST MEN1 INS GAST CHGA
21 dumping syndrome 30.6 SST INS GAST
22 pituitary tumors 30.6 SST MEN1 IGF1 GH1
23 von hippel-lindau syndrome 30.6 RET MEN1 CHGA
24 hyperparathyroidism 30.6 RET MEN1 GAST CHGA CALCA
25 duodenal somatostatinoma 30.6 SST MEN1 INS
26 cholelithiasis 30.5 SST INS GAST
27 gastric ulcer 30.5 SST GAST CALCA
28 potter's syndrome 30.5 INS IGF2 IGF1
29 mixed ductal-endocrine carcinoma 30.5 SYP GAST CHGA
30 ectopic cushing syndrome 30.5 SYP SST RET
31 hydrocephalus 30.5 VIP SYP SST IGF1
32 pancreatoblastoma 30.5 SYP SST CHGA
33 gastritis 30.5 SST GAST CHGA CCKBR
34 rare tumor 30.5 SST RET
35 adenoma 30.5 SST RET MEN1 IGF1 GH1 CHGA
36 adrenal cortical carcinoma 30.4 SYP MEN1 IGF2 CHGA
37 gangliocytoma 30.4 SYP GHRH CHGA
38 lung oat cell carcinoma 30.4 SYP CHGA CALCA
39 atrophic gastritis 30.4 SST MEN1 GAST CHGA
40 duodenal ulcer 30.4 SST INS GAST CALCA
41 duodenal gastrinoma 30.4 SST MEN1 GAST CHGA
42 gastrointestinal stromal tumor 30.3 SYP SST MEN1 IGF2 CHGA
43 short bowel syndrome 30.3 SST IGF1 GH1 GAST
44 thyroiditis 30.3 RET INS CALCA
45 bone resorption disease 30.3 INS IGF1 CALCA
46 merkel cell carcinoma 30.3 SYP SST CHGA
47 hyperglycemia 30.3 SST INS IGF1 IAPP GH1 GCGR
48 insulin-like growth factor i 30.3 SST INS IGF2 IGF1 GHRH GH1
49 small cell carcinoma 30.3 SYP GAST CHGA CALCA
50 hyperandrogenism 30.2 INS IGF2 IGF1 GH1

Graphical network of the top 20 diseases related to Islet Cell Tumor:



Diseases related to Islet Cell Tumor

Symptoms & Phenotypes for Islet Cell Tumor

MGI Mouse Phenotypes related to Islet Cell Tumor:

45 (show all 14)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.36 BRCA2 CCKBR CHGA GAST GCGR GHRH
2 behavior/neurological MP:0005386 10.35 BRCA2 CCKBR GCGR GHRH IGF2 IGF2R
3 growth/size/body region MP:0005378 10.35 BRCA2 CCKBR CHGA GCGR GHRH IAPP
4 endocrine/exocrine gland MP:0005379 10.34 BRCA2 CCKBR CHGA GAST GCGR GHRH
5 cardiovascular system MP:0005385 10.26 CCKBR CHGA IGF1 IGF2 IGF2R INS
6 immune system MP:0005387 10.25 BRCA2 CCKBR GAST GHRH IAPP IGF1
7 digestive/alimentary MP:0005381 10.21 BRCA2 CCKBR GAST GCGR IGF2 INS
8 mortality/aging MP:0010768 10.21 BRCA2 CHGA GAST GCGR IGF1 IGF2
9 nervous system MP:0003631 10.07 BRCA2 CCKBR CHGA GHRH IGF1 IGF2
10 liver/biliary system MP:0005370 9.95 CCKBR GCGR GHRH IGF2 IGF2R INS
11 neoplasm MP:0002006 9.87 BRCA2 GAST GCGR IGF1 MEN1 PALB2
12 normal MP:0002873 9.81 BRCA2 GCGR IGF1 IGF2R INS RET
13 renal/urinary system MP:0005367 9.56 CCKBR CHGA GCGR IGF1 IGF2 IGF2R
14 reproductive system MP:0005389 9.4 BRCA2 CHGA GCGR GHRH IGF1 IGF2

Drugs & Therapeutics for Islet Cell Tumor

Drugs for Islet Cell Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 208)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
2
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 5310940 9887054 6857599 43805
3
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Thiotepa Approved, Investigational Phase 3 52-24-4 5453
6
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
7
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
8
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
9
Cobalt Approved, Experimental Phase 3 7440-48-4 104729
10
leucovorin Approved Phase 3 58-05-9 6006 143
11
Levoleucovorin Approved, Investigational Phase 3 68538-85-2
12
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
13
Donepezil Approved Phase 3 120014-06-4 3152
14
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
15
Fluorouracil Approved Phase 3 51-21-8 3385
16
Streptozocin Approved, Investigational Phase 3 18883-66-4 29327
17
lanreotide Approved Phase 2, Phase 3 108736-35-2
18
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
19
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
20
Lenograstim Approved, Investigational Phase 3 135968-09-1
21
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
22
Isotretinoin Approved Phase 3 4759-48-2 5538 5282379
23
Somatostatin Approved, Investigational Phase 3 51110-01-1, 38916-34-6 53481605
24
Sunitinib Approved, Investigational Phase 3 341031-54-7, 557795-19-4 5329102
25
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
26
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
27
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 68-26-8, 11103-57-4, 22737-96-8 445354 9904001
28
Tretinoin Approved, Investigational, Nutraceutical Phase 3 302-79-4 5538 444795
29 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
30
Emodepside Investigational, Vet_approved Phase 3 155030-63-0
31 Immunosuppressive Agents Phase 3
32
Liposomal doxorubicin Phase 3 31703
33 Antimitotic Agents Phase 3
34 Etoposide phosphate Phase 3
35 Antirheumatic Agents Phase 3
36 Hormones Phase 3
37 Calcium, Dietary Phase 3
38 Central Nervous System Depressants Phase 3
39 Neurotransmitter Agents Phase 3
40 Liver Extracts Phase 3
41 Cola Phase 3
42 Vitamin B9 Phase 3
43 Folate Phase 3
44 Protective Agents Phase 3
45 Antidotes Phase 3
46 Vitamin B Complex Phase 3
47 Folic Acid Antagonists Phase 3
48 Analgesics Phase 3
49 Nootropic Agents Phase 3
50 Cholinergic Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 188)
# Name Status NCT ID Phase Drugs
1 Clinical Effectiveness of Serum Chromogranin A (CgA) Levels on Diagnostic Relevance, Response After Surgical Resection and Recurrence of Pancreatic Endocrine Tumors (PET) Unknown status NCT02759718 Phase 4
2 A SINGLE-ARM OPEN-LABEL INTERNATIONAL MULTI-CENTER STUDY OF THE EFFICACY AND SAFETY OF SUNITINIB MALATE (SU011248, SUTENT (REGISTERED)) IN PATIENTS WITH PROGRESSIVE ADVANCED METASTATIC WELL-DIFFERENTIATED UNRESECTABLE PANCREATIC NEUROENDOCRINE TUMORS Completed NCT01525550 Phase 4 sunitinib
3 Phase IV, Open-label, Multi-center, Single-arm Study of the Safety and Efficacy of Everolimus (Afinitor) in Adult Patients With Local Advanced or Metastatic, Well Differentiated Progressive Pancreatic Neuroendocrine Tumors (pNET) in China. Active, not recruiting NCT02842749 Phase 4 everolimus
4 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Unknown status NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
5 Phase II Prospective Study of Sequential Myeloablative Chemotherapy With Stem Cell Rescue for the Treatment of Selected High Risk CNS Tumors and Recurrent CNS Tumors Unknown status NCT00179803 Phase 2, Phase 3
6 EUROPEAN INTERGROUP COOPERATIVE EWING'S SARCOMA STUDY [EICESS 92] Unknown status NCT00002516 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;mesna;vincristine sulfate
7 Dose Intensive Chemotherapy for Children Less Than Ten Years of Age Newly-Diagnosed With Malignant Brain Tumors: A Pilot Study of Two Alternative Intensive Induction Chemotherapy Regimens, Followed by Consolidation With Myeloablative Chemotherapy (Thiotepa and Carboplatin, With or Without Etoposide) and Autologous Stem Cell Rescue [HEAD START III] Unknown status NCT00392886 Phase 3 carboplatin;cisplatin;cyclophosphamide;etoposide;methotrexate;temozolomide;thiotepa;vincristine sulfate
8 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)
9 A Randomized Double-blind Phase III Study of RAD001 10 mg/d Plus Best Supportive Care Versus Placebo Plus Best Supportive Care in the Treatment of Patients With Advanced Pancreatic Neuroendocrine Tumor (NET) Completed NCT00510068 Phase 3 Everolimus;Everolimus Placebo
10 Phase III Double Blind, Placebo Controlled Study of Donepezil in the Irradiated Brain Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo
11 A Phase III Randomized Trial for the Treatment of Newly Diagnosed Supratentorial PNET and High Risk Medulloblastoma in Children <36 Months Old With Intensive Induction Chemotherapy With Methotrexate Followed by Consolidation With Stem Cell Rescue Versus the Same Therapy Without Methotrexate Completed NCT00336024 Phase 3 etoposide;cyclophosphamide;cisplatin;carboplatin;thiotepa;methotrexate;leucovorin calcium;vincristine sulfate
12 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
13 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
14 18F-DOPA PET Imaging: an Evaluation of Biodistribution and Safety Recruiting NCT03042416 Phase 3 18F-DOPA
15 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
16 An International Prospective Study on Clinically Standard-risk Medulloblastoma in Children Older Than 3 to 5 Years With Low-risk Biological Profile (PNET 5 MB-LR) or Average-risk Biological Profile (PNET 5 MB-SR) Recruiting NCT02066220 Phase 2, Phase 3 Reduced-intensity maintenance chemotherapy;Maintenance chemotherapy
17 Randomized Open Label Study to Compare the Efficacy and Safety of Everolimus Followed by Chemotherapy With Streptozotocin- Fluorouracilo (STZ-5FU) Upon Progression or the Reverse Sequence, in Advanced Progressive Pancreatic NETs (pNETs) Recruiting NCT02246127 Phase 3 Drug: Everolimus;STZ-5FU
18 Efficacy of Carboplatin Administered Concomitantly With Radiation and Isotretinoin as a Pro-Apoptotic Agent in Other Than Average Risk Medulloblastoma/PNET Patients Active, not recruiting NCT00392327 Phase 3 Carboplatin;Cisplatin;Cyclophosphamide;Isotretinoin;Vincristine Sulfate
19 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
20 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
21 Activity and Safety of Everolimus in Combination With Octreotide LAR and Metformin in Patients With Advanced Pancreatic Well-differentiated Neuroendocrine Tumors (pWDNETs): a Phase II, Open, Monocentric, Prospective Study Unknown status NCT02294006 Phase 2 Everolimus plus Octreotide LAR plus Metformin
22 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
23 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.
24 Phase II Single Arm Trial With Combination of Everolimus and Letrozole in Treatment of Platinum Resistant Relapse or Refractory or Persistent Ovarian Cancer/Endometrial Cancer (CRAD001CUS242T) Unknown status NCT02188550 Phase 2 everolimus and letrozole
25 Phase II Study of Recombinant Anti-tumor and Anti-virus Protein for Injection to Treat Advanced Neuroendocrine Tumors Unknown status NCT02455596 Phase 2 Recombinant anti-tumor and anti-virus protein for injection (Novaferon)
26 Treatment Protocol for High-Risk PNET Brain Tumors in Children With Surgery, Sequential Chemotherapy, Conventional and High-Dose With Peripheral Blood Stem Cell Transplantation and Radiation Therapy Unknown status NCT00180791 Phase 2 Etoposide, carboplatin, melphalan, cisplatin, thiotepa
27 Phase II Randomized Study of Docetaxel With or Without Low-dose, Short Course Sunitinib in the Treatment of Advanced Solid Tumors Unknown status NCT01803503 Phase 2 Docetaxel;Sunitinib
28 177Lutetium-DOTA-Octreotate Therapy in Somatostatin Receptor-Expressing Neuroendocrine Neoplasms Unknown status NCT01237457 Phase 2 177Lu-DOTATATE
29 A Phase Ib, Open-label Study to Evaluate RAD001 as Monotherapy Treatment in Chinese Patients With Advanced Pulmonary Neuroendocrine Tumor Unknown status NCT01175096 Phase 1, Phase 2 RAD001 (everolimus, Afinitor®)
30 A Phase II Trial of Concurrent Sunitinib, Temozolomide and Radiation Therapy Followed by Adjuvant Temozolomide for Newly Diagnosed Glioblastoma Patients With an Unmethylated MGMT Gene Promoter Unknown status NCT02928575 Phase 2 Sunitinib;Temozolomide
31 A Prospective, Multicentre Trial on the Value of 18F-FET PET in the Post-therapeutic Evaluation of Childhood Brain Tumours Unknown status NCT03216148 Phase 2
32 Phase II Randomized Multicenter Study of Everolimus as Maintenance Therapy for Metastatic Neuroendocrine Carcinoma With Pulmonary or Gastroenteropancreatic Origin Unknown status NCT02687958 Phase 2 Everolimus
33 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
34 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
35 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)
36 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
37 A Phase II Study Of Sunitinib In Patients With Progressive Advanced/Metastatic Well-Differentiated Pancreatic Neuroendocrine Tumors Completed NCT01121562 Phase 2 Sunitinib
38 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
39 An Open Label, Stratified, Single-arm Phase II Study of Everolimus in Patients With Advanced Pancreatic Neuroendocrine Tumor (NET) After Failure of Cytotoxic Chemotherapy Completed NCT00363051 Phase 2 Everolimus 10 mg;Octreotide Depot
40 Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization for Metastatic Gastrointestinal Neuroendocrine Tumors Completed NCT00434109 Phase 2 Sunitinib malate
41 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
42 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
43 A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors Completed NCT01024387 Phase 2 AMG 479
44 Phase II Study of Everolimus (RAD001, Afinitor®) for Children With Recurrent or Progressive Ependymoma Completed NCT02155920 Phase 2 Everolimus
45 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
46 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
47 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
48 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
49 A Phase II Trial to Assess the Activity and Safety of Palbociclib in Patients With Well and Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors (pNET) Completed NCT02806648 Phase 2 Palbociclib
50 A Ph 2 Study to Investigate the Safety and Activity of Fosbretabulin Tromethamine (CA4P) in the Treatment of Well-Differentiated, Low-to-Intermediate-Grade Unresectable, Recurrent or Metastatic PNET or GI-NET Neuroendocrine Tumors/Carcinoid With Elevated Biomarkers Completed NCT02132468 Phase 2 fosbretabulin tromethamine

Search NIH Clinical Center for Islet Cell Tumor

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Dacarbazine

Cochrane evidence based reviews: adenoma, islet cell

Genetic Tests for Islet Cell Tumor

Anatomical Context for Islet Cell Tumor

The Foundational Model of Anatomy Ontology organs/tissues related to Islet Cell Tumor:

19
Pancreatic Islet Cells

MalaCards organs/tissues related to Islet Cell Tumor:

40
Pancreas, Brain, Pancreatic Islet, Liver, Pituitary, Thyroid, Lymph Node

Publications for Islet Cell Tumor

Articles related to Islet Cell Tumor:

(show top 50) (show all 1434)
# Title Authors PMID Year
1
Use of the tyrosine kinase inhibitor sunitinib in a patient with von Hippel-Lindau disease: targeting angiogenic factors in pheochromocytoma and other von Hippel-Lindau disease-related tumors. 54 61
19017755 2009
2
Insulin and somatostatin releasing islet cell tumor caused hypoglycemia. 54 61
11334393 2001
3
Islet cell tumor in von Hippel-Lindau disease. 54 61
9617866 1998
4
Multihormone-producing islet cell tumor of the pancreas associated with somatostatin-immunoreactive amyloid: immunohistochemical and immunoelectron microscopic studies. 54 61
9500779 1998
5
Pancreatic islet cell tumor producing vasoactive intestinal polypeptide and calcitonin. 54 61
9494586 1997
6
Absence of RET proto-oncogene mutations in a father and son with pheochromocytoma and pancreatic islet cell tumor. 54 61
9179691 1997
7
The glucagonoma syndrome. Clinical and pathologic features in 21 patients. 54 61
8606627 1996
8
PTHrP-mediated hypercalcemia in a calcitonin-producing islet cell tumor. 54 61
15251589 1995
9
Comparison of chromogranin A and pancreastatin levels in plasma of patients with pancreatic islet cell tumor. 54 61
7630318 1995
10
Production and secretion of chromogranin A and pancreastatin by the human pancreatic carcinoma cell line QGP-1N on stimulation with carbachol. 54 61
7800852 1994
11
Pancreastatin producing cell line from human pancreatic islet cell tumor. 54 61
2159299 1990
12
Ectopic Corticotropin-Releasing Hormone-Secreting Pancreatic Neuroendocrine Tumor: Excellent Response of Liver Metastases to Peptide Receptor Radionuclide Therapy as Demonstrated by 68Ga-DOTATOC and 18F-FDG PET/CT Imaging. 61
31714276 2020
13
Loss of nectin-3 expression as a marker of tumor aggressiveness in pancreatic neuroendocrine tumor. 61
31855317 2020
14
CT-Radiomic Approach to Predict G1/2 Nonfunctional Pancreatic Neuroendocrine Tumor. 61
32037260 2020
15
Temozolomide Alone or Combined with Capecitabine for the Treatment of Advanced Pancreatic Neuroendocrine Tumor. 61
31071715 2020
16
Neuroendocrine tumor with diarrhea: not always the usual suspects - a case report of metastatic calcitoninoma with literature review. 61
31900071 2020
17
[Use of a somatostatin analog to improve a patient's condition and the subsequent diagnosis of pancreatic VIPoma:a case report]. 61
31941861 2020
18
Alpha-fetoprotein producing pancreatic neuroendocrine tumor. 61
31999348 2020
19
A case of primary pancreatic schwannoma diagnosed by endoscopic ultrasound-fine needle aspiration. 61
31983049 2020
20
Two well-differentiated pancreatic neuroendocrine tumor mouse models. 61
31160716 2020
21
Metformin Inhibits Proliferation and Tumor Growth of QGP-1 Pancreatic Neuroendocrine Tumor Cells by Inducing Cell Cycle Arrest and Apoptosis. 61
31892560 2020
22
Spontaneous rupture of a non-functioning pancreatic neuroendocrine tumor A case report of a rare cause of acute abdomen. 61
32020903 2020
23
The impact of surgery for metastatic pancreatic neuroendocrine tumor: a contemporary evaluation matching for chromogranin a level. 61
31239188 2020
24
A patient with a pancreatic neuroendocrine tumor and multiple liver metastases achieved a long-term partial response to third-line streptozocin treatment. 61
31950018 2020
25
A growth model of neuroendocrine tumor surrogates and the efficacy of a novel somatostatin-receptor-guided antibody-drug conjugate: Perspectives on clinical response? 61
31543319 2020
26
Predictors of Recurrence and Survival in Patients With Surgically Resected Pancreatic Neuroendocrine Tumors. 61
32011530 2020
27
Insulinoma-associated protein 1 (INSM1) is a robust marker for identifying and grading pancreatic neuroendocrine tumors. 61
31977134 2020
28
miR-431 Promotes Metastasis of Pancreatic Neuroendocrine Tumors by Targeting DAB2 Interacting Protein, a Ras GTPase Activating Protein Tumor Suppressor. 61
31953039 2020
29
PET/MRI enables simultaneous in vivo quantification of β-cell mass and function. 61
31903128 2020
30
[Effects of Akkermansia muciniphila on the Proliferation, Apoptosis and Insulin Secretion of Rat Islet Cell Tumor Cells]. 61
31950783 2020
31
Impact of perioperative blood transfusion on survival in pancreatic neuroendocrine tumor patients: analysis from the US Neuroendocrine Study Group. 61
31806388 2019
32
Parallel Signaling through IRE1α and PERK Regulates Pancreatic Neuroendocrine Tumor Growth and Survival. 61
31672843 2019
33
Mixed serous-neuroendocrine neoplasm of the pancreas: A case report and review of the literature. 61
31832417 2019
34
Splenic cord capillary hemangioma with non-islet cell tumor hypoglycemia: a case report. 61
31842913 2019
35
ASO Author Reflections: Pancreatic Neuroendocrine Tumor Recurrence and Survival Predicted by Ki67. 61
30519760 2019
36
Carboplatin-related acute interstitial nephritis in a patient with pancreatic neuroendocrine tumor. 61
31834568 2019
37
Imaging and implications of tumor thrombus in abdominal malignancies: reviewing the basics. 61
31696267 2019
38
Evaluating the ACS NSQIP Risk Calculator in Primary Pancreatic Neuroendocrine Tumor: Results from the US Neuroendocrine Tumor Study Group. 61
30941685 2019
39
Tumor-infiltrating platelets predict postoperative recurrence and survival in resectable pancreatic neuroendocrine tumor. 61
31749595 2019
40
PROINSULIN-PREDOMINANT PANCREATIC NEUROENDOCRINE TUMOR-INDUCED HYPOGLYCEMIA AFTER ROUX-EN-Y GASTRIC BYPASS SURGERY. 61
31967066 2019
41
Bilateral breast metastasis from pancreatic neuroendocrine tumor: A diagnostic challenge. 61
31270897 2019
42
Evaluation of the Site and Frequency of Lymph Node Metastasis with Non-Functioning Pancreatic Neuroendocrine Tumor. 61
31734661 2019
43
Ampullary Adenocarcinoma with Incidental Pancreatic Neuroendocrine Tumor: Report of an Extremely Rare Case and Review of Literature. 61
31886078 2019
44
Recognizing intrapancreatic accessory spleen via EUS: Interobserver variability. 61
31417068 2019
45
A Clinicopathologic and Molecular Update of Pancreatic Neuroendocrine Neoplasms With a Focus on the New World Health Organization Classification. 61
31509453 2019
46
Deep learning for World Health Organization grades of pancreatic neuroendocrine tumors on contrast-enhanced magnetic resonance images: a preliminary study. 61
31555998 2019
47
Pixel-to-Pixel Learning With Weak Supervision for Single-Stage Nucleus Recognition in Ki67 Images. 61
30802845 2019
48
Robotic resection of the uncinate process of the pancreas. 61
30467703 2019
49
Validation of the 8th AJCC Cancer Staging System for Pancreas Neuroendocrine Tumors Using Korean Nationwide Surgery Database. 61
30999719 2019
50
Non-islet cell tumor-induced hypoglycemia in the setting of metastatic intracranial hemangiopericytoma: case report and review of the literature. 61
31599173 2019

Variations for Islet Cell Tumor

ClinVar genetic disease variations for Islet Cell Tumor:

6 ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 BRCA2 NM_000059.3(BRCA2):c.994dup (p.Ile332fs)duplication Pathogenic 52925 rs80359777 13:32906602-32906603 13:32332465-32332466
2 PALB2 NM_024675.3(PALB2):c.778C>T (p.Gln260Ter)SNV Pathogenic 560018 rs1555461627 16:23647089-23647089 16:23635768-23635768

Copy number variations for Islet Cell Tumor from CNVD:

7 (show all 16)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 36717 1 79269825 120225489 Loss DDA3 Pancreatic endocrine tumor
2 38231 10 1 77700000 Gain RET Pancreatic endocrine tumor
3 40010 10 123100000 127500000 Loss DMBT1 Pancreatic endocrine tumor
4 45931 10 75266020 135228726 Loss PTEN Pancreatic endocrine tumor
5 49582 11 108093559 108239826 Loss ATM Pancreatic endocrine tumor
6 49994 11 112500000 114500000 Loss PPP2R1B Pancreatic endocrine tumor
7 117037 17 70900000 81195210 Loss FAS Pancreatic endocrine tumor
8 151222 20 18623480 62342703 Gain BCAS1 Pancreatic endocrine tumor
9 162982 22 23484416 48053047 Loss CHEK2 Pancreatic endocrine tumor
10 166156 3 1 91700000 Indels Pancreatic endocrine tumor
11 176562 3 49525778 51884070 Loss IFRD2 Pancreatic endocrine tumor
12 179191 3 8700000 16400000 Loss FANCD2 Endocrine pancreatic tumor
13 191665 5 1 46100000 Gain TERT Pancreatic endocrine tumor
14 206334 6 139000000 149000000 Loss Endocrine pancreatic tumor
15 225336 7 45020224 55392724 Gain EGFR Pancreatic endocrine tumor
16 236915 8 18983547 53650298 Loss BNIP3L Pancreatic endocrine tumor

Expression for Islet Cell Tumor

Search GEO for disease gene expression data for Islet Cell Tumor.

Pathways for Islet Cell Tumor

Pathways related to Islet Cell Tumor according to GeneCards Suite gene sharing:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.88 VIP SST RET MEN1 INS IGF2
2
Show member pathways
13.39 VIP SST IAPP GHRH GH1 GCGR
3
Show member pathways
12.84 INS IGF2R IGF2 IGF1 GHRH GH1
4
Show member pathways
12.2 IAPP GHRH GCGR CALCA
5 12.09 VIP SYP RET PCSK1 CALCA
6
Show member pathways
11.97 PCSK1 INS IGF1 GH1
7
Show member pathways
11.56 IGF2R IGF2 IGF1 GHRH GH1
8 11.45 IGF2 IGF1 GH1
9 11.22 INS IGF2 IGF1
10 11.19 VIP IAPP GHRH GCGR CALCA
11 10.28 VIP GAST
12 9.96 GAST CCKBR

GO Terms for Islet Cell Tumor

Cellular components related to Islet Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.7 VIP SST INS IGF2 IGF1 IAPP
2 transport vesicle GO:0030133 9.46 PCSK1 INS IGF2R CHGA
3 terminal bouton GO:0043195 9.43 SYP GHRH CALCA
4 extracellular space GO:0005615 9.4 SST PCSK1 INS IGF2R IGF2 IGF1

Biological processes related to Islet Cell Tumor according to GeneCards Suite gene sharing:

(show all 22)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.13 VIP RET INS IGF2R IGF1 IAPP
2 positive regulation of cell proliferation GO:0008284 9.97 VIP INS IGF2 IGF1 GHRH CCKBR
3 positive regulation of protein kinase B signaling GO:0051897 9.84 RET INS IGF2 IAPP
4 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.76 IGF2 IGF1 GH1
5 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.75 INS IGF1 GH1
6 regulation of blood pressure GO:0008217 9.73 GCGR CHGA CALCA
7 positive regulation of MAPK cascade GO:0043410 9.72 RET INS IGF2 IGF1 IAPP
8 response to heat GO:0009408 9.7 SST IGF1 CALCA
9 positive regulation of multicellular organism growth GO:0040018 9.69 IGF2 GHRH GH1
10 positive regulation of mitotic nuclear division GO:0045840 9.65 INS IGF2 IGF1
11 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.65 VIP IAPP GHRH GCGR CALCA
12 positive regulation of blood vessel diameter GO:0097755 9.63 VIP INS CALCA
13 cellular protein metabolic process GO:0044267 9.63 MEN1 INS IGF2 IGF1 IAPP CALCA
14 insulin-like growth factor receptor signaling pathway GO:0048009 9.61 IGF2R IGF1
15 negative regulation of bone resorption GO:0045779 9.6 IAPP CALCA
16 inner cell mass cell proliferation GO:0001833 9.59 PALB2 BRCA2
17 positive regulation of glycogen biosynthetic process GO:0045725 9.58 INS IGF2 IGF1
18 negative regulation of blood vessel diameter GO:0097756 9.57 INS CHGA
19 amylin receptor signaling pathway GO:0097647 9.55 IAPP CALCA
20 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 9.54 IGF1 GHRH GH1
21 cell-cell signaling GO:0007267 9.43 SST PCSK1 INS IAPP GHRH CALCA
22 G protein-coupled receptor signaling pathway GO:0007186 9.32 VIP SST INS IGF2R IAPP GHRH

Molecular functions related to Islet Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.8 SYP PCSK1 INS IGF2R IAPP CALCA
2 insulin receptor binding GO:0005158 9.33 INS IGF2 IGF1
3 peptide hormone receptor binding GO:0051428 9.32 VIP GHRH
4 hormone activity GO:0005179 9.28 VIP SST INS IGF2 IGF1 IAPP
5 insulin-like growth factor receptor binding GO:0005159 9.13 INS IGF2 IGF1

Sources for Islet Cell Tumor

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
Content
Loading form....