MCID: SMT003
MIFTS: 52

Somatostatinoma

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

Aliases & Classifications for Somatostatinoma

MalaCards integrated aliases for Somatostatinoma:

Name: Somatostatinoma 12 73 20 58 54 44 15 17 70
Somatostatin-Secreting Pancreatic Neoplasm 20
Somatomedin-Secreting Carcinoid 20
Pancreatic Endocrine Carcinoma 70
Somatostatin Cell Neoplasm 12
Pancreatic Somatostatinoma 70
Malignant Islet Cell Tumor 20
Ampullary Somatostatinoma 20
Carcinoid Somatostatinoma 20
Somatostatin Cell Tumour 12

Characteristics:

Orphanet epidemiological data:

58
somatostatinoma
Prevalence: <1/1000000 (Europe); Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


Summaries for Somatostatinoma

MalaCards based summary : Somatostatinoma, also known as somatostatin-secreting pancreatic neoplasm, is related to jejunal somatostatinoma and pancreatic somatostatinoma, and has symptoms including diarrhea An important gene associated with Somatostatinoma is SST (Somatostatin), and among its related pathways/superpathways are Peptide ligand-binding receptors and Signaling by GPCR. The drugs Fentanyl and Fluorouracil have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, liver and thyroid, and related phenotypes are nausea and vomiting and constipation

Wikipedia : 73 Somatostatinomas are a tumor of the delta cells of the endocrine pancreas that produces somatostatin.... more...

Related Diseases for Somatostatinoma

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

(show top 50) (show all 317)
# Related Disease Score Top Affiliating Genes
1 jejunal somatostatinoma 31.9 SST PPY
2 pancreatic somatostatinoma 31.6 SST PPY NF1 CALCA
3 cholelithiasis 31.0 SST INS GAST
4 rare tumor 30.5 SST ACTC1
5 glucose intolerance 30.4 SST INS GCG
6 duodenal somatostatinoma 30.4 SST PPY NF1 MEN1 INS GAST
7 microcystic adenoma 30.3 ENO2 CHGA
8 glucagonoma 30.3 VIP SST GAST CHGA
9 bile acid malabsorption, primary 30.1 SST GCG
10 gastric ulcer 30.0 SST GAST CALCA
11 goiter 30.0 SST INS CALCA
12 ampulla of vater benign neoplasm 29.9 SST ENO2 CHGA
13 peptic ulcer disease 29.9 SST MEN1 GAST
14 merkel cell carcinoma 29.9 SST ENO2 CHGA
15 horseshoe kidney 29.8 ENO2 CHGA ACTC1
16 neurofibromatosis, type i 29.7 SST NF1 MEN1 ENO2
17 von hippel-lindau syndrome 29.7 NF1 MEN1 CHGA
18 hyperinsulinism 29.6 SST INS GCG
19 neurilemmoma 29.6 NF1 ENO2 ACTC1
20 tuberous sclerosis 29.5 SST NF1 INS ENO2 CHGA
21 rhabdomyosarcoma 29.5 NF1 INS ENO2 CHGA ACTC1
22 parathyroid adenoma 29.5 MEN1 CHGA CALCA
23 teratoma 29.4 SST INS GCG ENO2 CHGA ACTC1
24 carcinoid syndrome 29.3 SST MEN1 GHRH ENO2 CHGA CALCA
25 multiple endocrine neoplasia 29.2 VIP SST NF1 MEN1 GAST CHGA
26 adenoma 29.2 SSTR2 SST MEN1 GHRH CHGA
27 hemangioma 29.1 MEN1 GAST ENO2 CHGA ACTC1
28 carcinoid tumors, intestinal 29.1 SSTR2 SST MEN1 GAST ENO2 CHGA
29 neuroendocrine carcinoma 29.1 SST MEN1 GAST ENO2 CHGA CALCA
30 zollinger-ellison syndrome 29.0 SST MEN1 GHRH GAST CHGA
31 gastrointestinal stromal tumor 28.9 SST NF1 MEN1 EPAS1 ENO2 CHGA
32 paraganglioma 28.9 SST NF1 MEN1 EPAS1 ENO2 CHGA
33 thyroid gland medullary carcinoma 28.8 SSTR2 SST MEN1 GAST CHGA CALCA
34 thyroid carcinoma, familial medullary 28.8 SSTR2 SST MEN1 GAST CHGA CALCA
35 gastrinoma 28.6 VIP SSTR2 SST MEN1 INS GAST
36 diabetes mellitus 28.6 SST MEN1 INS GCG GAST CHGA
37 insulinoma 28.5 SSTR2 SST MEN1 INS GCG GAST
38 hyperinsulinemic hypoglycemia 28.5 SST PPY MEN1 INS GCG ENO2
39 neuroendocrine tumor 28.4 VIP SSTR2 SST MEN1 GHRH GCG
40 pheochromocytoma 28.3 VIP SST NF1 MEN1 EPAS1 ENO2
41 pancreatic endocrine carcinoma 28.3 SSTR2 SST PPY MEN1 INS GCG
42 islet cell tumor 28.1 VIP SST PPY MEN1 INS GHRH
43 multiple endocrine neoplasia, type i 27.8 VIP SST PPY NF1 MEN1 INS
44 multiple paragangliomas associated with polycythemia 11.0
45 neurofibromatosis 10.5
46 acidophil adenoma 10.4 SST INS
47 autoimmune atrophic gastritis 10.4 GAST CHGA
48 duodenal benign neoplasm 10.4 SST CHGA
49 mixed ductal-endocrine carcinoma 10.4 GAST CHGA
50 gastric antral vascular ectasia 10.4 SST GAST

Comorbidity relations with Somatostatinoma via Phenotypic Disease Network (PDN):


Pancreatic Cancer

Graphical network of the top 20 diseases related to Somatostatinoma:



Diseases related to Somatostatinoma

Symptoms & Phenotypes for Somatostatinoma

Human phenotypes related to Somatostatinoma:

58 31 (show all 36)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
2 constipation 58 31 frequent (33%) Frequent (79-30%) HP:0002019
3 diabetes mellitus 58 31 frequent (33%) Frequent (79-30%) HP:0000819
4 hepatomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0002240
5 anorexia 58 31 frequent (33%) Frequent (79-30%) HP:0002039
6 chronic fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012432
7 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
8 neoplasm of the small intestine 58 31 frequent (33%) Frequent (79-30%) HP:0100833
9 steatorrhea 58 31 frequent (33%) Frequent (79-30%) HP:0002570
10 neoplasm of the pancreas 58 31 frequent (33%) Frequent (79-30%) HP:0002894
11 diarrhea 58 31 frequent (33%) Frequent (79-30%) HP:0002014
12 poor appetite 58 31 frequent (33%) Frequent (79-30%) HP:0004396
13 hypochromic microcytic anemia 58 31 frequent (33%) Frequent (79-30%) HP:0004840
14 episodic abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002574
15 gallbladder dysfunction 58 31 frequent (33%) Frequent (79-30%) HP:0005609
16 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
17 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
18 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214
19 intrahepatic cholestasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001406
20 extrahepatic cholestasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012334
21 intermittent jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0001046
22 lack of bowel sounds 58 31 occasional (7.5%) Occasional (29-5%) HP:0030145
23 increased circulating gonadotropin level 58 31 very rare (1%) Very rare (<4-1%) HP:0000837
24 hypercalcemia 58 31 very rare (1%) Very rare (<4-1%) HP:0003072
25 growth hormone excess 58 31 very rare (1%) Very rare (<4-1%) HP:0000845
26 subcutaneous lipoma 58 31 very rare (1%) Very rare (<4-1%) HP:0001031
27 medullary thyroid carcinoma 58 31 very rare (1%) Very rare (<4-1%) HP:0002865
28 parathyroid adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0002897
29 adrenocortical adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0008256
30 pituitary adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0002893
31 primary hyperparathyroidism 58 31 very rare (1%) Very rare (<4-1%) HP:0008200
32 increased circulating prolactin concentration 58 31 very rare (1%) Very rare (<4-1%) HP:0000870
33 increased circulating cortisol level 31 very rare (1%) HP:0003118
34 abnormality of the thyroid gland 58 Very rare (<4-1%)
35 hypercortisolism 58 Very rare (<4-1%)
36 abnormality of abdomen morphology 58 Occasional (29-5%)

UMLS symptoms related to Somatostatinoma:


diarrhea

MGI Mouse Phenotypes related to Somatostatinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.81 ACTC1 CHGA EPAS1 GHRH INS MEN1
2 homeostasis/metabolism MP:0005376 9.7 ACTC1 CHGA EPAS1 GAST GHRH INS
3 nervous system MP:0003631 9.32 ACTC1 CHGA ENO2 EPAS1 GHRH INS

Drugs & Therapeutics for Somatostatinoma

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

(show top 50) (show all 85)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
3
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2 149436
4
leucovorin Approved Phase 2, Phase 3 58-05-9 6006
5
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 5310940 9887054 43805 6857599
6
Bevacizumab Approved, Investigational Phase 2, Phase 3 216974-75-3
7
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
8 Pancreatic Polypeptide Investigational Phase 2, Phase 3 59763-91-6
9 Cola Phase 3
10 Anesthetics Phase 3
11 Narcotics Phase 3
12 Analgesics, Opioid Phase 3
13 Analgesics Phase 3
14 Anesthetics, Intravenous Phase 3
15 Anesthetics, General Phase 3
16 Vitamin B9 Phase 2, Phase 3
17 Nutrients Phase 2, Phase 3
18 Micronutrients Phase 2, Phase 3
19 Trace Elements Phase 2, Phase 3
20 Protective Agents Phase 2, Phase 3
21 Vitamin B Complex Phase 2, Phase 3
22 Vitamins Phase 2, Phase 3
23 Folate Phase 2, Phase 3
24 Immunosuppressive Agents Phase 2, Phase 3
25 Antidotes Phase 2, Phase 3
26 Immunologic Factors Phase 2, Phase 3
27 Angiogenesis Inhibitors Phase 2, Phase 3
28 Antineoplastic Agents, Immunological Phase 2, Phase 3
29
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
30
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
31
Melphalan Approved Phase 2 148-82-3 4053 460612
32
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
33
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
34
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
35
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
36
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
37
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
38
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
39
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
40
Pancrelipase Approved, Investigational Phase 2 53608-75-6
41
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
42
Gemcitabine Approved Phase 2 95058-81-4 60750
43
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
44
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
45
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
46
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616
47
Vitamin A Approved, Nutraceutical, Vet_approved Phase 2 68-26-8, 11103-57-4 445354
48
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
49
Imetelstat Investigational Phase 2 868169-64-6
50 topoisomerase I inhibitors Phase 2

Interventional clinical trials:

(show all 34)
# Name Status NCT ID Phase Drugs
1 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
2 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Terminated NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
3 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
4 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
5 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
6 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
7 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
8 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
9 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
10 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Completed NCT02259725 Phase 2 regorafenib
11 Comparator Study of 68Ga-DOTATOC PET/CT With Octreoscan + High-resolution, Contrast-enhanced CT for Diagnosis and Staging in Neuroendocrine Tumors and Other Somatostatin Receptor Positive Tumors Completed NCT01869725 Phase 2
12 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
13 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
14 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
15 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
16 A Phase 2 Study of Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 Capecitabine;Temozolomide
17 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
18 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
19 Phase II Study of Depsipeptide in Metastatic Neuroendocrine Tumors Terminated NCT00084461 Phase 2 romidepsin
20 Phase II Evaluation of Real-Time, Pharmacokinetically Guided Everolimus in Patients With Hormone Receptor Positive Breast Cancer, Pancreatic Neuroendocrine Tumors (PNET), and Renal Cell Carcinoma Terminated NCT02273752 Phase 2 Everolimus
21 A Randomized, Double-Blinded, Placebo-Controlled Phase II Study of Adjuvant Everolimus Following the Resection of Metastatic Pancreatic Neuroendocrine Tumors to the Liver Terminated NCT02031536 Phase 2 everolimus
22 Phase II Study of Antineoplastons A10 and AS2-1 in Patients With Metastatic or Incurable Neuroendocrine Tumors Withdrawn NCT00003514 Phase 2 antineoplaston A10;antineoplaston AS2-1
23 Phase II Study of Vatalanib and Octreotide in Patients With Progressive Low-Grade Neuroendocrine Tumors Withdrawn NCT00227773 Phase 2 octreotide acetate;vatalanib
24 Phase II Study of Dovitinib (TKI-258) in Progressive, Well-Differentiated Pancreatic Neuroendocrine Tumors With and Without Prior VEGF-Inhibitor Therapy Withdrawn NCT02108782 Phase 2 dovitinib lactate
25 A Phase I, Pharmacokinetic, and Biologic Correlative Study of R115777 (NSC 702818) and Herceptin in Patients With Advanced Cancer Completed NCT00005842 Phase 1 tipifarnib
26 A Phase I Study of Intraperitoneal Oxaliplatin Alone and in Combination With Intraperitoneal Floxuridine and Leucovorin in Patients With Advanced Metastatic Cancer Confined to the Peritoneal Cavity Completed NCT00005860 Phase 1 floxuridine;leucovorin calcium;oxaliplatin
27 A Phase I, Open-Label, Maximum Tolerated Single-Cycle and Four-Cycle Dose-Finding Study to Evaluation the Safety and Tolerability of 90Y-SMT 487 Administered by Intravenous Infusion to Subjects With Refractory Somatostatin-Receptor Positive Tumors Completed NCT00006368 Phase 1
28 A Phase I Trial of Herceptin and Interleukin-12 Completed NCT00004074 Phase 1
29 Phase I Study of Anti-IGF-1R Monoclonal Antibody, IMC-A12, and mTOR Inhibitor, Everolimus, in Advanced Low to Intermediate Grade Neuroendocrine Carcinoma Completed NCT01204476 Phase 1 Everolimus;Octreotide Acetate
30 A Phase I Trial of the mTOR Inhibitor RAD001 in Combination With VEGF Receptor Tyrosine Kinase Inhibitor PTK787/ZK 222584 in Patients With Advanced Solid Tumors Completed NCT00655655 Phase 1 everolimus;vatalanib
31 A Phase I Study of [111In-DTPA-D-Phe]-Octreotide in Patients With Refractory Malignancies Expressing Somatostatin Receptors Terminated NCT00002947 Phase 1
32 A Phase 1 Study of Veliparib (ABT-888) in Combination With Capecitabine and Temozolomide in Advanced Well-Differentiated Neuroendocrine Tumors Withdrawn NCT02831179 Phase 1 Capecitabine;Temozolomide;Veliparib
33 Clinical and Psychometric Validation of a Disease-Specific Questionnaire Module in Assessing the Quality of Life of Patients With G.I.-Related Neuroendocrine Tumours Unknown status NCT00454376
34 Treatment of Patients With Hepatic Neuroendocrine Metastases Using Drug-Eluting Bead Embolization Terminated NCT00730483 PVA microporous hydrospheres/doxorubicin hydrochloride

Search NIH Clinical Center for Somatostatinoma

Inferred drug relations via UMLS 70 / NDF-RT 51 :


Dacarbazine

Cochrane evidence based reviews: somatostatinoma

Genetic Tests for Somatostatinoma

Anatomical Context for Somatostatinoma

MalaCards organs/tissues related to Somatostatinoma:

40
Pancreas, Liver, Thyroid, Pituitary, Small Intestine, Endothelial, Pancreatic Islet
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Somatostatinoma:
# Tissue Anatomical CompartmentCell Relevance
1 Pancreas Head of Pancreas Affected by disease

Publications for Somatostatinoma

Articles related to Somatostatinoma:

(show top 50) (show all 333)
# Title Authors PMID Year
1
Mixed endocrine somatostatinoma of the ampulla of vater associated with a neurofibromatosis type 1: a case report and review of the literature. 61 54
20065557 2010
2
Two synchronous somatostatinomas of the duodenum and pancreatic head in one patient. 61 54
19998510 2009
3
Somatostatinoma: clinico-pathological features of three cases and literature reviewed. 61 54
17645474 2008
4
[Somatostatinoma of the Vater's papilla in a patient with von Recklinghausen's disease]. 61 54
17438969 2007
5
Somatostatinoma: collision with neurofibroma and ultrastructural features. 61 54
16874660 2006
6
Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion. 61 54
16105971 2005
7
Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case. 61 54
15851380 2005
8
Duodenal somatostatinoma associated with Von Recklinghausen's disease. 61 54
15619018 2004
9
Gallbladder motility in obesity, diabetes mellitus and coeliac disease. 54 61
12974503 2003
10
A small, incidentally detected pancreatic somatostatinoma: report of a case. 61 54
12560911 2003
11
Case report: somatostatin producing teratoma, causing rapidly alternating extreme hyperglycemia and hypoglycemia, and ovarian somatostatinoma. 54 61
12200764 2002
12
Effect of somatostatin on human gallbladder motility: an in vitro study. 61 54
10087534 1999
13
Somatostatin receptor scintigraphy of malignant somatostatinoma with indium-111-pentetreotide. 61 54
9189135 1997
14
Use of calcium provocative test in the diagnosis of gastroenteropancreatic endocrine tumors. 61 54
9063035 1996
15
[Pancreatic and duodenal somatostatinoma. Two clinico-pathologic entities]. 54 61
9172623 1996
16
Carcinoid syndrome due to a malignant somatostatinoma. 54 61
7494779 1995
17
Somatostatinoma: atypical presentation of a rare pancreatic tumor. 61 54
7733095 1995
18
Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas. 54 61
7745981 1995
19
Clinical features of duodenal somatostatinomas. 61 54
8256221 1993
20
A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF-oma, hypercalcitoninaemia and intractable peptic ulceration. 61 54
1356665 1992
21
Somatostatin-producing neuroendocrine tumor of the ampulla (ampullary somatostatinoma). Evidence of prosomatostatin production. 54 61
1371903 1992
22
von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary thyroid carcinoma and diffuse adrenal medullary hyperplasia. 54 61
1686137 1991
23
Amyloid protein in somatostatinoma differs from human islet amyloid polypeptide. 54 61
2000701 1991
24
Parallel in vivo and in vitro detection of functional somatostatin receptors in human endocrine pancreatic tumors: consequences with regard to diagnosis, localization, and therapy. 61 54
2168430 1990
25
[Malignant somatostatinoma--diagnosis after 6 years]. 61 54
1974023 1990
26
Pancreatic somatostatinoma: a case report and review of the literature. 54 61
1969977 1990
27
Developmental vascular malformations in EPAS1 gain-of-function syndrome. 61
33497361 2021
28
Endoscopic Ampullectomy of a Somatostatinoma. 61
31954200 2021
29
Functional significance of germline EPAS1 variants. 61
33300499 2021
30
C-Terminal, but Not Intact, FGF23 and EPO Are Strongly Correlatively Elevated in Patients With Gain-of-Function Mutations in HIF2A: Clinical Evidence for EPO Regulating FGF23. 61
33049075 2021
31
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging. 61
33305819 2020
32
Somatostatinoma: Beyond neurofibromatosis type 1 (Review). 61
32905002 2020
33
Somatostatinoma and Neurofibromatosis Type 1-A Case Report and Review of the Literature. 61
32825782 2020
34
Neuraxial dysraphism in EPAS1-associated syndrome due to improper mesenchymal transition. 61
32337341 2020
35
Involvement of the γ1 subunit of the large-conductance Ca2+-activated K+ channel in the proliferation of human somatostatinoma cells. 61
32178873 2020
36
Clinical manifestations of Pacak-Zhuang syndrome in a male pediatric patient. 61
31876082 2020
37
Vascular Changes in the Retina and Choroid of Patients With EPAS1 Gain-of-Function Mutation Syndrome. 61
31876943 2020
38
Clinical Characteristics and Management of Functional Pancreatic Neuroendocrine Neoplasms: A Single Institution 20-Year Experience with 286 Patients. 61
33204258 2020
39
Chiari Malformation Type 1 in EPAS1-Associated Syndrome. 61
31185588 2019
40
A Transgenic Mouse Model of Pacak⁻Zhuang Syndrome with An Epas1 Gain-of-Function Mutation. 61
31091718 2019
41
Somatostatinoma Presented as Double-Duct Sign. 61
31210994 2019
42
Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature. 61
30635305 2019
43
Coexistence of neurofibromatosis type 1 with multiple malignant neoplasia. 61
30431740 2018
44
Somatostatinoma Masquerading as Chronic Pancreatitis. 61
29521948 2018
45
Mixed mucinous adenocarcinoma and somatostatinoma of the ampulla of Vater associated with neurofibromatosis type 1. 61
28693748 2017
46
Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes. 61
28336782 2017
47
[Diabetes mellitus secondary to an endocrine pathology : when to think about it ?] 61
28639759 2017
48
Course of Aggressive Somatotroph, Corticotroph and Mammotroph Tumors under Temozolomide; Report of Three Cases and Review of the Literature. 61
28608351 2017
49
Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome. 61
27679736 2016
50
Correlative Light- and Electron Microscopy Using Quantum Dot Nanoparticles. 61
27584907 2016

Variations for Somatostatinoma

Expression for Somatostatinoma

Search GEO for disease gene expression data for Somatostatinoma.

Pathways for Somatostatinoma

GO Terms for Somatostatinoma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.61 VIP SST PPY INS GHRH GCG
2 extracellular space GO:0005615 9.32 SST PPY INS GHRH GCG GAST

Biological processes related to Somatostatinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 9.98 VIP SSTR2 PPY NF1 INS GAST
2 cell-cell signaling GO:0007267 9.67 SST INS GHRH CALCA
3 neuropeptide signaling pathway GO:0007218 9.58 SSTR2 PPY CALCA
4 response to food GO:0032094 9.46 GHRH GAST
5 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.46 VIP GHRH GCG CALCA
6 feeding behavior GO:0007631 9.43 PPY GCG CALCA
7 negative regulation of blood vessel diameter GO:0097756 9.32 INS CHGA
8 G protein-coupled receptor signaling pathway GO:0007186 9.28 VIP SSTR2 SST PPY INS GHRH
9 positive regulation of blood vessel diameter GO:0097755 9.13 VIP INS CALCA

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

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.23 VIP SST PPY INS GHRH GCG
2 peptide hormone receptor binding GO:0051428 9.16 VIP GHRH
3 neuropeptide hormone activity GO:0005184 9.13 VIP PPY GHRH

Sources for Somatostatinoma

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