PNET
MCID: PNC119
MIFTS: 48

Pancreatic Neuroendocrine Tumor (PNET)

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

Aliases & Classifications for Pancreatic Neuroendocrine Tumor

MalaCards integrated aliases for Pancreatic Neuroendocrine Tumor:

Name: Pancreatic Neuroendocrine Tumor 53 59 37 17
Neuroendocrine Tumor of Pancreas 53 59 6
Well-Differentiated Neuroendocrine Neoplasm of Pancreas 53 59
Well-Differentiated Pancreatic Neuroendocrine Neoplasm 53 59
Well-Differentiated Nen of Pancreas 53 59
Well-Differentiated Pancreatic Nen 53 59
Islet Cell Tumor 53 72
Pancreatic Net 53 59
Pnet 53 59
Well Differentiated Pancreatic Endocrine Tumor 72
Pancreatic Neuroendocrine Neoplasm 53
Pancreatic Endocrine Tumor 53

Characteristics:

Orphanet epidemiological data:

59
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:



External Ids:

KEGG 37 H00045
ICD10 via Orphanet 34 E16.8
UMLS via Orphanet 73 C0242363
Orphanet 59 ORPHA97253
UMLS 72 C0242363 C1337011

Summaries for Pancreatic Neuroendocrine Tumor

NIH Rare Diseases : 53 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 : Pancreatic Neuroendocrine Tumor, also known as neuroendocrine tumor of pancreas, is related to meningioma, radiation-induced and tumor predisposition syndrome. An important gene associated with Pancreatic Neuroendocrine Tumor is BRCA2 (BRCA2 DNA Repair Associated), and among its related pathways/superpathways are Sterol Regulatory Element-Binding Proteins (SREBP) signalling and Human Thyroid Stimulating Hormone (TSH) signaling pathway. The drugs Doxorubicin and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, brain and liver, and related phenotype is nervous system.

KEGG : 37
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.

Wikipedia : 75 Pancreatic neuroendocrine tumors (PanNETs, PETs, or PNETs), often referred to as "islet cell tumors", or... more...

Related Diseases for Pancreatic Neuroendocrine Tumor

Diseases related to Pancreatic Neuroendocrine Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 452)
# Related Disease Score Top Affiliating Genes
1 meningioma, radiation-induced 30.1 MKI67 MEG3
2 tumor predisposition syndrome 29.4 PALB2 BRCA2
3 glioma 29.0 PIK3CA MEG3 BRCA2
4 glioblastoma 28.7 PIK3CA MTOR MEG3 BRCA2
5 pancreatic cancer 28.5 PIK3CA PALB2 MTOR MEG3 BRCA2
6 endometrial cancer 27.9 PIK3CA MTOR MKI67 MEG3 BRCA2
7 breast cancer 27.3 PIK3CA PALB2 MTOR MKI67 MEG3 BRCA2
8 islet cell tumor 12.7
9 non-functioning pancreatic endocrine tumor 12.7
10 pheochromocytoma--islet cell tumor syndrome 12.7
11 functioning pancreatic endocrine tumor 12.5
12 ewing sarcoma 12.1
13 von hippel-lindau syndrome 12.0
14 pineoblastoma 12.0
15 neuroendocrine tumor 11.9
16 insulinoma 11.9
17 glucagonoma 11.7
18 vipoma 11.7
19 mahvash disease 11.7
20 gcgr-related hyperglucagonemia 11.7
21 functioning neuroendocrine tumor of pancreas 11.7
22 non-functioning neuroendocrine tumor of pancreas 11.7
23 serotonin-producing neuroendocrine tumor of pancreas 11.7
24 central nervous system primitive neuroectodermal tumor 11.6
25 medulloblastoma 11.5
26 supratentorial primitive neuroectodermal tumor 11.5
27 intracranial primitive neuroectodermal tumor 11.4
28 cerebral primitive neuroectodermal tumor 11.4
29 insulinomatosis and diabetes mellitus 11.4
30 neuroendocrine carcinoma of pancreas 11.4
31 adult central nervous system primitive neuroectodermal neoplasm 11.1
32 childhood central nervous system primitive neuroectodermal neoplasm 11.1
33 cerebellopontine angle primitive neuroectodermal 11.1
34 spinal cord primitive neuroectodermal neoplasm 11.1
35 childhood supratentorial embryonal tumor, not otherwise specified 11.1
36 ewing's family of tumors 10.8
37 multiple endocrine neoplasia 10.8
38 multiple endocrine neoplasia, type i 10.8
39 neuroendocrine carcinoma 10.7
40 pancreatic adenocarcinoma 10.5
41 hypoglycemia 10.5
42 adenocarcinoma 10.5
43 zollinger-ellison syndrome 10.5
44 gastrinoma 10.5
45 pancreatic ductal adenocarcinoma 10.4
46 renal cell carcinoma, nonpapillary 10.4
47 pancreatic endocrine carcinoma 10.4
48 pheochromocytoma 10.3
49 exanthem 10.3
50 adrenal gland pheochromocytoma 10.3

Graphical network of the top 20 diseases related to Pancreatic Neuroendocrine Tumor:



Diseases related to Pancreatic Neuroendocrine Tumor

Symptoms & Phenotypes for Pancreatic Neuroendocrine Tumor

MGI Mouse Phenotypes related to Pancreatic Neuroendocrine Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 9.1 ALCAM BRCA2 INA MTOR PALB2 PIK3CA

Drugs & Therapeutics for Pancreatic Neuroendocrine Tumor

Drugs for Pancreatic Neuroendocrine Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 205)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
2
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
3
Etoposide Approved Phase 3 33419-42-0 36462
4
Thiotepa Approved, Investigational Phase 3 52-24-4 5453
5
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
6
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
7
Cobalt Approved, Experimental Phase 3 7440-48-4 104729
8
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
9
leucovorin Approved Phase 3 58-05-9 143 6006
10
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
11
Levoleucovorin Approved, Investigational Phase 3 68538-85-2
12
Donepezil Approved Phase 3 120014-06-4 3152
13
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
14
Fluorouracil Approved Phase 3 51-21-8 3385
15
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
16
Streptozocin Approved, Investigational Phase 3 18883-66-4 29327
17
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
18
Lenograstim Approved, Investigational Phase 3 135968-09-1
19
Sargramostim Approved, Investigational Phase 3 83869-56-1, 123774-72-1
20
Isotretinoin Approved Phase 3 4759-48-2 5282379 5538
21
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
22
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
23
Tretinoin Approved, Investigational, Nutraceutical Phase 3 302-79-4 5538 444795
24
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 22737-96-8, 11103-57-4, 68-26-8 9904001 445354
25 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
26
Emodepside Investigational, Vet_approved Phase 3 155030-63-0
27 Immunosuppressive Agents Phase 3
28
Liposomal doxorubicin Phase 3 31703
29 Antineoplastic Agents, Phytogenic Phase 3
30 Etoposide phosphate Phase 3
31 Tubulin Modulators Phase 3
32 Antimitotic Agents Phase 3
33 Antirheumatic Agents Phase 3
34 Hormones Phase 3
35 Central Nervous System Depressants Phase 3
36 Neurotransmitter Agents Phase 3
37 Liver Extracts Phase 3
38 Calcium, Dietary Phase 3
39 Folic Acid Antagonists Phase 3
40 Nucleic Acid Synthesis Inhibitors Phase 3
41 Vitamin B9 Phase 3
42 Folate Phase 3
43 Vitamin B Complex Phase 3
44 Antidotes Phase 3
45 Protective Agents Phase 3
46 Analgesics Phase 3
47 Nootropic Agents Phase 3
48 Peripheral Nervous System Agents Phase 3
49 Cholinesterase Inhibitors Phase 3
50 Cholinergic Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 150)
# 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 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
5 EUROPEAN INTERGROUP COOPERATIVE EWING'S SARCOMA STUDY [EICESS 92] Unknown status NCT00002516 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;mesna;vincristine sulfate
6 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
7 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
8 Phase III Double Blind, Placebo Controlled Study of Donepezil in the Irradiated Brain Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo
9 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
10 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
11 18F-DOPA PET Imaging: an Evaluation of Biodistribution and Safety Recruiting NCT03042416 Phase 3 18F-DOPA
12 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
13 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
14 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
15 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
16 Activity and Safety of Everolimus in Combination With Octreotide LAR and Metformin in Patients With Advanced Pancreatic Well-differentiated Neuroendocrine Tumors (pWDNETs): a Phase II, Open, Monocentric, Prospective Study Unknown status NCT02294006 Phase 2 Everolimus plus Octreotide LAR plus Metformin
17 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
18 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
19 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)
20 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
21 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
22 177Lutetium-DOTA-Octreotate Therapy in Somatostatin Receptor-Expressing Neuroendocrine Neoplasms Unknown status NCT01237457 Phase 2 177Lu-DOTATATE
23 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®)
24 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
25 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
26 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
27 Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor Completed NCT01845675 Phase 2 temozolomide or dacarbazine-based chemotherapy, endostatin
28 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)
29 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
30 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
31 Phase II Study of Everolimus (RAD001, Afinitor®) for Children With Recurrent or Progressive Ependymoma Completed NCT02155920 Phase 2 Everolimus
32 Use of 68Ga-DOTATATE PET Scanning for Diagnosis and Treatment of Metastatic Neuroendocrine Tumors Completed NCT01396382 Phase 2
33 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
34 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
35 Feasibility of Using Concurrent Carboplatin and Reduced Dose Craniospinal Radiation (24Gy) for Metastatic Medulloblastoma, High-Risk Supratentorial PNET and Metastatic PNET Completed NCT01542736 Phase 2 Carboplatin;Vincristine
36 A Phase II Study of Pemetrexed in Children With Recurrent Malignancies Completed NCT00520936 Phase 2 pemetrexed
37 Accuracy and Clinical Impact of 68-Ga-labeled Octreotide Analogues PET in Diagnosis and Staging of Duodenal-pancreatic Neuroendocrine Tumours; Proposal of a Multicenter, Prospective Clinical Trial Completed NCT01673906 Phase 2 Diagnostic work up
38 A Five-Tier, Phase 2 Open-Label Study of IMC-A12 Administered as a Single Agent Every 2 Weeks in Patients With Previously-Treated, Advanced or Metastatic Soft Tissue and Ewing's Sarcoma/PNET Completed NCT00668148 Phase 2
39 A Phase II Study of R115777 (Zarnestra) (NSC # 702818, IND# 58,359) in Children With Recurrent or Progressive: High Grade Glioma, Medulloblastoma/PNET or Brainstem Glioma Completed NCT00070525 Phase 2 tipifarnib
40 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
41 A Phase II Trial of Mutation-Targeted Therapy With Sunitinib or Everolimus in Patients With Advanced Low-or Intermediate Grade Neuroendocrine Tumors of the Gastrointestinal Tract and Pancreas With or Without Cytoreductive Surgery Completed NCT02315625 Phase 2 Sunitinib;Everolimus
42 Rollover for Study OX4218s, A P2 Study to Investigate Safety and Activity of Fosbretabulin Tromethamine in the Treatment of Well-Differentiated, Low-to-Intermediate-Grade Unresectable, Recurrent or Metastatic PNET or GI-NET With Elevated Biomarkers Completed NCT02279602 Phase 2 fosbretabulin
43 Phase II Trial of Irinotecan in Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
44 Phase II Study of Methotrexate, Mechlorethamine, Vincristine, Prednisone, and Procarbazine (MMOPP) as Primary Therapy in Infants or Young Children With Primitive Neuroectodermal Tumors or High-Grade Astrocytoma Completed NCT00002463 Phase 2 MOPP Regimen;Leucovorin Calcium;Mechlorethamine Hydrochloride;Methotrexate;Prednisone;Procarbazine Hydrochloride;Vincristine Sulfate
45 Phase II Multicenter, Open-label, Clinical and Pharmacokinetic Study of Zalypsis® (PM00104) in Patients With Unresectable Locally Advanced and/or Metastatic Ewing Family of Tumors (EFT) Progressing After at Least One Prior Line of Chemotherapy Completed NCT01222767 Phase 2 Zalypsis
46 A Phase II Trial of Preradiation Multiagent Chemotherapy for Adults With "Poor Risk" Medulloblastoma, PNET, and Disseminated Ependymoma Completed NCT00003309 Phase 2 cisplatin;cyclophosphamide;etoposide;vincristine sulfate
47 Molecular Biology and Phase II Study of Lapatinib (GW572016) in Pediatric Patients With Recurrent or Refractory Medulloblastoma, Malignant Glioma or Ependymoma Completed NCT00095940 Phase 1, Phase 2 lapatinib ditosylate
48 CAMP 013:- Tandem Thiotepa Regimen For Selected Malignant Gliomas:1) Primary Or Recurrent Glioblastoma Multiforme (GBM); and 2) Recurrent Anaplastic Astrocytomas (AA), Oligodendrogliomas (O), Oligoastrocytomas (OA), Ependymomas And Primitive Neuroectodermal Tumors (PNET) That Have Either Progressed After Primary Therapy Or Are Refractory To Standard Chemotherapy Completed NCT00008008 Phase 2 cyclophosphamide;thiotepa
49 Dose Intensive Chemotherapy for Patients Greater Than or Equal To 10 Years of Age With Newly Diagnosed High Stage Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumors (PNET) and Ependymoma: A Feasibility Study of an Intensive Induction Chemotherapy Regimen Followed by Standard Irradiation Completed NCT00006258 Phase 2 cisplatin;cyclophosphamide;etoposide;methotrexate;vincristine sulfate
50 A Phase II Trial of Rebeccamycin Analogue (NSC #655649) in Children With Solid Tumors Completed NCT00006102 Phase 2 becatecarin

Search NIH Clinical Center for Pancreatic Neuroendocrine Tumor

Genetic Tests for Pancreatic Neuroendocrine Tumor

Anatomical Context for Pancreatic Neuroendocrine Tumor

MalaCards organs/tissues related to Pancreatic Neuroendocrine Tumor:

41
Pancreas, Brain, Liver, Lymph Node, Spleen, Bone, Breast

Publications for Pancreatic Neuroendocrine Tumor

Articles related to Pancreatic Neuroendocrine Tumor:

(show top 50) (show all 655)
# Title Authors PMID Year
1
Well-differentiated Pancreatic Neuroendocrine Tumor in a Patient With Familial Atypical Multiple Mole Melanoma Syndrome (FAMMM). 38
31261289 2019
2
Pilot study of rapid MR pancreas screening for patients with BRCA mutation. 38
30689033 2019
3
Letter to the Editor: Intrapancreatic Accessory Spleen Masquerading as a Pancreatic Neuroendocrine Tumor. 38
31152350 2019
4
Significance of Lymph Node Metastasis in Resectable Well-differentiated Pancreatic Neuroendocrine Tumor. 38
31268980 2019
5
Differentiation of atypical non-functional pancreatic neuroendocrine tumor and pancreatic ductal adenocarcinoma using CT based radiomics. 38
31307634 2019
6
Minimally invasive versus open distal pancreatectomy for pancreatic neuroendocrine tumors: An analysis from the U.S. neuroendocrine tumor study group. 38
31001868 2019
7
Recognizing intrapancreatic accessory spleen via EUS: Interobserver variability. 38
31417068 2019
8
Pancreatic schwannoma, an extremely rare and challenging entity: Report of two cases and review of literature. 38
31153779 2019
9
Air Embolism during Upper Endoscopy: A Case Report. 38
30862154 2019
10
Efficacy of the tryptophan hydroxylase inhibitor telotristat on growth and serotonin secretion in 2D and 3D cultured pancreatic neuroendocrine tumor cells. 38
31319410 2019
11
Characteristics and Outcomes of Pancreatic Cancer by Histological Subtypes. 38
31210663 2019
12
68Ga-DOTATATE Uptake in Pancreatic Metastasis of Renal Cell Carcinoma Mimicking Pancreatic Neuroendocrine Tumor. 38
31306201 2019
13
Bilateral breast metastasis from pancreatic neuroendocrine tumor: A diagnostic challenge. 38
31270897 2019
14
Differentiating intrapancreatic accessory spleen from a pancreatic neuroendocrine tumor or metastasis by the "bridge sign". 38
31249165 2019
15
Leveraging machine learning techniques for predicting pancreatic neuroendocrine tumor grades using biochemical and tumor markers. 38
31367620 2019
16
Rare breast and subcutaneous metastases from pancreatic neuroendocrine tumor: a case report. 38
31296241 2019
17
High-Grade Gastrointestinal Neuroendocrine Carcinoma Management and Outcomes: A National Cancer Database Study. 38
30482824 2019
18
Diagnostic utility of Gallium-68-somatostatin receptor PET/CT in ectopic ACTH-secreting tumors: a systematic literature review and single-center clinical experience. 38
31236798 2019
19
Endoscopic ultrasound-guided radiofrequency ablation for pancreatic neuroendocrine tumor. 38
31053252 2019
20
First-in-human phase 1 study of novel dUTPase inhibitor TAS-114 in combination with S-1 in Japanese patients with advanced solid tumors. 38
30511200 2019
21
Concurrent Adult-Onset Diffuse β-Cell Nesidioblastosis and Pancreatic Neuroendocrine Tumor: A Case Report and Review of the Literature. 38
31244364 2019
22
Two well-differentiated pancreatic neuroendocrine tumor mouse models. 38
31160716 2019
23
Tumor thrombus in the venous drainage pathways of primary, recurrent and metastatic disease on routine oncologic imaging studies: beyond hepatocellular and renal cell carcinomas. 38
30844299 2019
24
Pancreatic Neuroendocrine Tumor in a Young Child With Tuberous Sclerosis Complex 1. 38
31187078 2019
25
Utility of cytomorphology in distinguishing solid pseudopapillary neoplasm of pancreas from pancreatic neuroendocrine tumor with emphasis on nuclear folds and nuclear grooves. 38
30677247 2019
26
Time-trend and recurrence analysis of pancreatic neuroendocrine tumors. 38
31252400 2019
27
Correction to: Phase II study of sunitinib in Japanese patients with unresectable or metastatic, well-differentiated pancreatic neuroendocrine tumor. 38
30903344 2019
28
Pre-therapy Somatostatin Receptor-Based Heterogeneity Predicts Overall Survival in Pancreatic Neuroendocrine Tumor Patients Undergoing Peptide Receptor Radionuclide Therapy. 38
30014345 2019
29
The impact of surgery for metastatic pancreatic neuroendocrine tumor: a contemporary evaluation matching for chromogranin a level. 38
31239188 2019
30
Genomic characterization of a well-differentiated grade 3 pancreatic neuroendocrine tumor. 38
31160355 2019
31
Locally Recurrent Well-Differentiated Nonfunctioning Pancreatic Neuroendocrine Tumor Requiring Re-excision Including Portal Vein Resection. 38
28965184 2019
32
Intrapancreatic Accessory Spleen Along With Hepatic Adenomas Mimicking Pancreatic Neuroendocrine Tumor Associated With Liver Metastasis. 38
31090665 2019
33
Clinical remission of an inoperable malignant insulinoma by the combination treatment with octreotide and everolimus. 38
31065475 2019
34
Function and clinical relevance of RHAMM isoforms in pancreatic tumor progression. 38
31072393 2019
35
Late metachronous isolated pancreatic metastasis from renal cell carcinoma mimicking a pancreatic neuroendocrine tumor. 38
30541739 2019
36
Microforceps-Assisted Diagnosis of Cystic Pancreatic Neuroendocrine Tumor. 38
31121996 2019
37
Surgery for Pancreatic Neuroendocrine Tumor G3 and Carcinoma G3 Should be Considered Separately. 38
30863939 2019
38
Central pancreatectomy for benign or low-grade malignant pancreatic lesions - A single-center retrospective analysis of 116 cases. 38
30527222 2019
39
UCHL1 loss alters the cell-cycle in metastatic pancreatic neuroendocrine tumors. 38
30689542 2019
40
Prognostic significance of Chromogranin A in small pancreatic neuroendocrine tumors. 38
30447803 2019
41
Evaluating the ACS NSQIP Risk Calculator in Primary Pancreatic Neuroendocrine Tumor: Results from the US Neuroendocrine Tumor Study Group. 38
30941685 2019
42
[A Case Report of Pancreatic Neuroendocrine Tumor Resected with the Preoperative Diagnosis of Gastrointestinal Stromal Tumor of the Jejunum]. 38
31164536 2019
43
Hepatocellular Carcinoma Mimicking Neuroendocrine Tumor Metastasis on 68Ga-DOTATATE PET/CT. 38
30688738 2019
44
Distinct clinicopathological and prognostic features of insulinoma with synchronous distant metastasis. 38
30850220 2019
45
Validation of the 8th AJCC Cancer Staging System for Pancreas Neuroendocrine Tumors Using Korean Nationwide Surgery Database. 38
30999719 2019
46
[A Resected Case of Tumorigenic Pancreatic Endocrine Cell Hyperplasia]. 38
30914610 2019
47
Ectopic corticotropin-releasing hormone syndrome caused by pancreatic neuroendocrine tumor localized by 68Ga-DOTATATE PET/CT. 38
30519868 2019
48
RABL6A inhibits tumor-suppressive PP2A/AKT signaling to drive pancreatic neuroendocrine tumor growth. 38
30721156 2019
49
Value of Texture Analysis of Intravoxel Incoherent Motion Parameters in Differential Diagnosis of Pancreatic Neuroendocrine Tumor and Pancreatic Adenocarcinoma. 38
30961774 2019
50
Clinicopathological features and prognosis factors for survival in elderly patients with pancreatic neuroendocrine tumor: A STROBE-compliant article. 38
30882623 2019

Variations for Pancreatic Neuroendocrine Tumor

ClinVar genetic disease variations for Pancreatic Neuroendocrine Tumor:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 PALB2 NM_024675.3(PALB2): c.778C> T (p.Gln260Ter) single nucleotide variant Pathogenic rs1555461627 16:23647089-23647089 16:23635768-23635768
2 BRCA2 NM_000059.3(BRCA2): c.994dup (p.Ile332fs) duplication Pathogenic rs80359777 13:32906609-32906609 13:32332472-32332472

Copy number variations for Pancreatic Neuroendocrine Tumor from CNVD:

7 (show all 16)
# CNVD ID Chromosom 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 Pancreatic Neuroendocrine Tumor

Search GEO for disease gene expression data for Pancreatic Neuroendocrine Tumor.

Pathways for Pancreatic Neuroendocrine Tumor

GO Terms for Pancreatic Neuroendocrine Tumor

Biological processes related to Pancreatic Neuroendocrine Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cardiac muscle contraction GO:0060048 9.37 PIK3CA MTOR
2 cell aging GO:0007569 9.32 MTOR BRCA2
3 negative regulation of macroautophagy GO:0016242 9.26 PIK3CA MTOR
4 inner cell mass cell proliferation GO:0001833 9.16 PALB2 BRCA2
5 anoikis GO:0043276 8.96 PIK3CA MTOR
6 multicellular organism growth GO:0035264 8.62 PALB2 MTOR

Sources for Pancreatic Neuroendocrine Tumor

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