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Small Intestinal Gastrin-Producing Neuroendocrine Tumor

MedGen UID:
475774
•Concept ID:
C3274141
•
Neoplastic Process
Synonyms: Small Intestinal G Cell NET; Small Intestinal Gastrin-Producing NET; Small Intestinal Gastrinoma

Definition

A gastrin-producing neuroendocrine tumor that arises from the small intestine. It is characterized by the presence of uniform cells that form pseudorosettes. The neoplastic cells have uniform nuclei and small amount of eosinophilic cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall Intestinal Gastrin-Producing Neuroendocrine Tumor

Professional guidelines

PubMed

Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies.
Shao QQ, Zhao BB, Dong LB, Cao HT, Wang WB
World J Gastroenterol 2019 Aug 28;25(32):4673-4681. doi: 10.3748/wjg.v25.i32.4673. PMID: 31528093Free PMC Article
Symptomatic and Radiological Response to 177Lu-DOTATATE for the Treatment of Functioning Pancreatic Neuroendocrine Tumors.
Zandee WT, Brabander T, Blažević A, Kam BLR, Teunissen JJM, Feelders RA, Hofland J, de Herder WW
J Clin Endocrinol Metab 2019 Apr 1;104(4):1336-1344. doi: 10.1210/jc.2018-01991. PMID: 30566620
Nuclear medicine in the detection and management of pancreatic islet-cell tumours.
Virgolini I, Traub-Weidinger T, Decristoforo C
Best Pract Res Clin Endocrinol Metab 2005 Jun;19(2):213-27. doi: 10.1016/j.beem.2004.09.001. PMID: 15763696

Recent clinical studies

Etiology

Recurrence after surgical resection of nonmetastatic sporadic gastrinoma: Which prognostic factors and surgical procedure?
Robin L, Sauvanet A, Walter T, Najah H, Falconi M, Pattou F, Gaujoux S; Gastrinoma Surgery Collaborative group
Surgery 2023 May;173(5):1144-1152. Epub 2023 Feb 11 doi: 10.1016/j.surg.2022.12.030. PMID: 36781315
Genome analysis identifies differences in the transcriptional targets of duodenal versus pancreatic neuroendocrine tumours.
Rico K, Duan S, Pandey RL, Chen Y, Chakrabarti JT, Starr J, Zavros Y, Else T, Katona BW, Metz DC, Merchant JL
BMJ Open Gastroenterol 2021 Nov;8(1) doi: 10.1136/bmjgast-2021-000765. PMID: 34750164Free PMC Article
Gastroenteropancreatic neuroendocrine tumors: Role of surgery.
Sauvanet A
Ann Endocrinol (Paris) 2019 Jun;80(3):175-181. Epub 2019 Apr 11 doi: 10.1016/j.ando.2019.04.009. PMID: 31079831
Proton pump inhibitor therapy and potential long-term harm.
Corleto VD, Festa S, Di Giulio E, Annibale B
Curr Opin Endocrinol Diabetes Obes 2014 Feb;21(1):3-8. doi: 10.1097/MED.0000000000000031. PMID: 24310148
Localisation and staging of gastrin producing tumours using cross-sectional imaging modalities.
Klose KJ, Heverhagen JT
Wien Klin Wochenschr 2007;119(19-20):588-92. doi: 10.1007/s00508-007-0886-0. PMID: 17985093

Diagnosis

Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies.
Shao QQ, Zhao BB, Dong LB, Cao HT, Wang WB
World J Gastroenterol 2019 Aug 28;25(32):4673-4681. doi: 10.3748/wjg.v25.i32.4673. PMID: 31528093Free PMC Article
Gastroenteropancreatic neuroendocrine tumors: Role of surgery.
Sauvanet A
Ann Endocrinol (Paris) 2019 Jun;80(3):175-181. Epub 2019 Apr 11 doi: 10.1016/j.ando.2019.04.009. PMID: 31079831
Proton pump inhibitor therapy and potential long-term harm.
Corleto VD, Festa S, Di Giulio E, Annibale B
Curr Opin Endocrinol Diabetes Obes 2014 Feb;21(1):3-8. doi: 10.1097/MED.0000000000000031. PMID: 24310148
The Zollinger-Ellison syndrome and mismeasurement of gastrin.
Rehfeld JF, Gingras MH, Bardram L, Hilsted L, Goetze JP, Poitras P
Gastroenterology 2011 May;140(5):1444-53. Epub 2011 Mar 23 doi: 10.1053/j.gastro.2011.01.051. PMID: 21315717
Gastrinoma--morphological aspects.
Klöppel G, Anlauf M
Wien Klin Wochenschr 2007;119(19-20):579-84. doi: 10.1007/s00508-007-0885-1. PMID: 17985091

Therapy

Symptomatic and Radiological Response to 177Lu-DOTATATE for the Treatment of Functioning Pancreatic Neuroendocrine Tumors.
Zandee WT, Brabander T, Blažević A, Kam BLR, Teunissen JJM, Feelders RA, Hofland J, de Herder WW
J Clin Endocrinol Metab 2019 Apr 1;104(4):1336-1344. doi: 10.1210/jc.2018-01991. PMID: 30566620
Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours).
Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J, Bałdys-Waligórska A, Bednarczuk T, Bolanowski M, Boratyn-Nowicka A, Borowska M, Cichocki A, Ćwikła JB, Falconi M, Foltyn W, Handkiewicz-Junak D, Hubalewska-Dydejczyk A, Jarząb B, Junik R, Kajdaniuk D, Kamiński G, Kolasińska-Ćwikła A, Kowalska A, Król R, Królicki L, Krzakowski M, Kunikowska J, Kuśnierz K, Lampe P, Lange D, Lewczuk-Myślicka A, Lewiński A, Lipiński M, Londzin-Olesik M, Marek B, Nasierowska-Guttmejer A, Nawrocki S, Nowakowska-Duława E, Pilch-Kowalczyk J, Rosiek V, Ruchała M, Siemińska L, Sowa-Staszczak A, Starzyńska T, Steinhof-Radwańska K, Sworczak K, Syrenicz A, Szawłowski A, Szczepkowski M, Wachuła E, Zajęcki W, Zemczak A, Zgliczyński W, Zieniewicz K
Endokrynol Pol 2017;68(2):79-110. doi: 10.5603/EP.2017.0015. PMID: 28597909
Proton pump inhibitor therapy and potential long-term harm.
Corleto VD, Festa S, Di Giulio E, Annibale B
Curr Opin Endocrinol Diabetes Obes 2014 Feb;21(1):3-8. doi: 10.1097/MED.0000000000000031. PMID: 24310148
Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours).
Kos-Kudła B, Blicharz-Dorniak J, Handkiewicz-Junak D, Jarząb B, Jarząb M, Kunikowska J, Kuśnierz K, Król R, Królicki L, Krzakowski M, Nasierowska-Guttmejer A, Nowakowska-Duława E, Patkowski W, Szawłowski AW; Consensus Conference; Polish Network of Neuroendocrine Tumours
Endokrynol Pol 2013;64(6):418-43. doi: 10.5603/EP.2013.0028. PMID: 24431116
Localisation and staging of gastrin producing tumours using cross-sectional imaging modalities.
Klose KJ, Heverhagen JT
Wien Klin Wochenschr 2007;119(19-20):588-92. doi: 10.1007/s00508-007-0886-0. PMID: 17985093

Prognosis

Recurrence after surgical resection of nonmetastatic sporadic gastrinoma: Which prognostic factors and surgical procedure?
Robin L, Sauvanet A, Walter T, Najah H, Falconi M, Pattou F, Gaujoux S; Gastrinoma Surgery Collaborative group
Surgery 2023 May;173(5):1144-1152. Epub 2023 Feb 11 doi: 10.1016/j.surg.2022.12.030. PMID: 36781315
Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: epidemiological changes in Germany.
Scherübl H, Streller B, Stabenow R, Herbst H, Höpfner M, Schwertner C, Steinberg J, Eick J, Ring W, Tiwari K, Zappe SM
World J Gastroenterol 2013 Dec 21;19(47):9012-9. doi: 10.3748/wjg.v19.i47.9012. PMID: 24379626Free PMC Article
Gastrinoma--morphological aspects.
Klöppel G, Anlauf M
Wien Klin Wochenschr 2007;119(19-20):579-84. doi: 10.1007/s00508-007-0885-1. PMID: 17985091
Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients.
Phan GQ, Yeo CJ, Hruban RH, Lillemoe KD, Pitt HA, Cameron JL
J Gastrointest Surg 1998 Sep-Oct;2(5):472-82. PMID: 9843608
Hormone-producing gastrointestinal tumors contain a high density of somatostatin receptors.
Reubi JC, Häcki WH, Lamberts SW
J Clin Endocrinol Metab 1987 Dec;65(6):1127-34. doi: 10.1210/jcem-65-6-1127. PMID: 2824549

Clinical prediction guides

Recurrence after surgical resection of nonmetastatic sporadic gastrinoma: Which prognostic factors and surgical procedure?
Robin L, Sauvanet A, Walter T, Najah H, Falconi M, Pattou F, Gaujoux S; Gastrinoma Surgery Collaborative group
Surgery 2023 May;173(5):1144-1152. Epub 2023 Feb 11 doi: 10.1016/j.surg.2022.12.030. PMID: 36781315
Gastroenteropancreatic neuroendocrine tumors: Role of surgery.
Sauvanet A
Ann Endocrinol (Paris) 2019 Jun;80(3):175-181. Epub 2019 Apr 11 doi: 10.1016/j.ando.2019.04.009. PMID: 31079831
Neuroendocrine Tumors (NETs) of the Minor Papilla/Ampulla: Analysis of 16 Cases Underlines Homology With Major Ampulla NETs and Differences From Extra-Ampullary Duodenal NETs.
Vanoli A, Albarello L, Uncini S, Fassan M, Grillo F, Di Sabatino A, Martino M, Pasquali C, Milanetto AC, Falconi M, Partelli S, Doglioni C, Schiavo-Lena M, Brambilla T, Pietrabissa A, Sessa F, Capella C, Rindi G, La Rosa S, Solcia E, Paulli M
Am J Surg Pathol 2019 Jun;43(6):725-736. doi: 10.1097/PAS.0000000000001234. PMID: 30913089
Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours).
Kos-Kudła B, Blicharz-Dorniak J, Handkiewicz-Junak D, Jarząb B, Jarząb M, Kunikowska J, Kuśnierz K, Król R, Królicki L, Krzakowski M, Nasierowska-Guttmejer A, Nowakowska-Duława E, Patkowski W, Szawłowski AW; Consensus Conference; Polish Network of Neuroendocrine Tumours
Endokrynol Pol 2013;64(6):418-43. doi: 10.5603/EP.2013.0028. PMID: 24431116
Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients.
Phan GQ, Yeo CJ, Hruban RH, Lillemoe KD, Pitt HA, Cameron JL
J Gastrointest Surg 1998 Sep-Oct;2(5):472-82. PMID: 9843608

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