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Pancreatic Somatostatin-Producing Neuroendocrine Tumor

MedGen UID:
1710142
•Concept ID:
C5235061
•
Neoplastic Process
Synonyms: Pancreatic Somatostatin Producing NET; Pancreatic Somatostatin Producing Neuroendocrine Tumor; Pancreatic Somatostatin Producing Tumor

Definition

A usually malignant, somatostatin producing neuroendocrine tumor, arising from the delta cells of the pancreas. It may or may not be associated with inappropriate secretion of somatostatin and an associated clinical syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic Somatostatin-Producing Neuroendocrine Tumor

Professional guidelines

PubMed

Diagnostic Management of Gastroenteropancreatic Neuroendocrine Neoplasms: Technique Optimization and Tips and Tricks for Radiologists.
Pellegrino F, Granata V, Fusco R, Grassi F, Tafuto S, Perrucci L, Tralli G, Scaglione M
Tomography 2023 Jan 27;9(1):217-246. doi: 10.3390/tomography9010018. PMID: 36828370Free PMC Article
Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Recent clinical studies

Etiology

Biomarkers in neuroendocrine neoplasms.
Zouli C, Zisimopoulou E, Chrisoulidou A
Hell J Nucl Med 2023 May-Aug;26 Suppl:44-48. PMID: 37658563
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.
Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
Anderson CW, Bennett JJ
Surg Oncol Clin N Am 2016 Apr;25(2):363-74. Epub 2016 Feb 18 doi: 10.1016/j.soc.2015.12.003. PMID: 27013370
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Diagnosis

EPAS1-related pheochromocytoma/paraganglioma.
Alzahrani AS, Alswailem M, Buffet A, Alghamdi B, Alobaid L, Alsagheir O, Al-Hindi H, Pacak K
Endocr Relat Cancer 2024 Aug 1;31(8) Epub 2024 Jun 10 doi: 10.1530/ERC-23-0303. PMID: 38767322
European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.
Hofland J, Falconi M, Christ E, Castaño JP, Faggiano A, Lamarca A, Perren A, Petrucci S, Prasad V, Ruszniewski P, Thirlwell C, Vullierme MP, Welin S, Bartsch DK
J Neuroendocrinol 2023 Aug;35(8):e13318. Epub 2023 Aug 14 doi: 10.1111/jne.13318. PMID: 37578384
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.
Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article
Pancreatic neuroendocrine tumors.
Batcher E, Madaj P, Gianoukakis AG
Endocr Res 2011;36(1):35-43. doi: 10.3109/07435800.2010.525085. PMID: 21226566

Therapy

Pharmacotherapeutic Management of Well-Differentiated Neuroendocrine Tumors in Older Patients: Current Status and Potential Therapies.
Crook C, Zhang YH, Li D
Drugs Aging 2022 Apr;39(4):257-269. Epub 2022 Mar 25 doi: 10.1007/s40266-022-00934-1. PMID: 35332446
Pancreatic neuroendocrine tumor producing vasopressin: A case report.
Li J, Zhang X, He Q, Feng W, Ding L, Wang Z, Yu H, Chen Q, Lu N, Xu D, Cui J
Medicine (Baltimore) 2021 Oct 8;100(40):e27453. doi: 10.1097/MD.0000000000027453. PMID: 34622867Free PMC Article
When and How to Use Somatostatin Analogues.
de Herder WW
Endocrinol Metab Clin North Am 2018 Sep;47(3):549-555. Epub 2018 Jul 11 doi: 10.1016/j.ecl.2018.04.010. PMID: 30098715
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article
Clinical and pathophysiological aspects of somatostatin and the gastrointestinal tract.
Lamers CB
Acta Endocrinol Suppl (Copenh) 1987;286:19-25. doi: 10.1530/acta.0.115s019. PMID: 2892334

Prognosis

Biomarkers in neuroendocrine neoplasms.
Zouli C, Zisimopoulou E, Chrisoulidou A
Hell J Nucl Med 2023 May-Aug;26 Suppl:44-48. PMID: 37658563
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.
Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
Anderson CW, Bennett JJ
Surg Oncol Clin N Am 2016 Apr;25(2):363-74. Epub 2016 Feb 18 doi: 10.1016/j.soc.2015.12.003. PMID: 27013370
Gastrointestinal hormones stimulate growth of Foregut Neuroendocrine Tumors by transactivating the EGF receptor.
Di Florio A, Sancho V, Moreno P, Delle Fave G, Jensen RT
Biochim Biophys Acta 2013 Mar;1833(3):573-82. Epub 2012 Dec 4 doi: 10.1016/j.bbamcr.2012.11.021. PMID: 23220008Free PMC Article
Pancreatic somatostatinoma.
Soldati T, Del Noce G, Garino M, Farina EC, De Paolis P, Balbo G
Panminerva Med 1990 Jul-Sep;32(3):141-4. PMID: 2077482

Clinical prediction guides

Effects of Ketoconazole on ACTH-Producing and Non-ACTH-Producing Neuroendocrine Tumor Cells.
Herrera-Martínez AD, Feelders RA, de Herder WW, Castaño JP, Gálvez Moreno MÁ, Dogan F, van Dungen R, van Koetsveld P, Hofland LJ
Horm Cancer 2019 Jun;10(2-3):107-119. Epub 2019 May 18 doi: 10.1007/s12672-019-00361-6. PMID: 31102172Free PMC Article
Health-related quality of life in well-differentiated metastatic gastroenteropancreatic neuroendocrine tumors.
Jiménez-Fonseca P, Carmona-Bayonas A, Martín-Pérez E, Crespo G, Serrano R, Llanos M, Villabona C, García-Carbonero R, Aller J, Capdevila J, Grande E; Spanish Neuroendocrine Tumor Group (GETNE)
Cancer Metastasis Rev 2015 Sep;34(3):381-400. doi: 10.1007/s10555-015-9573-1. PMID: 26245646
Biochemical markers for gastroenteropancreatic neuroendocrine tumours (GEP-NETs).
Kanakis G, Kaltsas G
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):791-802. doi: 10.1016/j.bpg.2012.12.006. PMID: 23582919
Periampullary pancreatic somatostatinoma.
House MG, Yeo CJ, Schulick RD
Ann Surg Oncol 2002 Nov;9(9):869-74. doi: 10.1007/BF02557523. PMID: 12417508
Distribution of somatostatin receptors in normal and tumor tissue.
Reubi JC, Kvols L, Krenning E, Lamberts SW
Metabolism 1990 Sep;39(9 Suppl 2):78-81. doi: 10.1016/0026-0495(90)90217-z. PMID: 2169575

Recent systematic reviews

Somatostatinoma of the Ampulla of Vater: A Systematic Review.
Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044

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