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Small Intestinal Somatostatin-Producing Neuroendocrine Tumor

MedGen UID:
236821
•Concept ID:
C1335992
•
Neoplastic Process
Synonyms: Small Intestinal Somatostatin Producing Tumor; Small Intestinal Somatostatin-Producing NET

Definition

A somatostatin-producing neuroendocrine tumor that arises from the small intestine. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

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

LONG-TERM FOLLOW-UP OF DUODENAL PAPILLARY SOMATOSTATINOMA TREATED BY ENDOSCOPIC PAPILLECTOMY.
Ardengh JC, Ardengh AO, Almeida AF, Machado MA
Arq Gastroenterol 2021 Jul-Sep;58(3):411-412. doi: 10.1590/S0004-2803.202100000-71. PMID: 34705982
Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.
Kumar T, Gupta B, Das P, Jain D, Jain HA, Madhusudhan KS, Dash NR, Gupta SD
Indian J Pathol Microbiol 2016 Jul-Sep;59(3):359-61. doi: 10.4103/0377-4929.188123. PMID: 27510677
Short-term results of 42 endoscopic ampullectomies: a single-center experience.
Jeanniard-Malet O, Caillol F, Pesenti C, Bories E, Monges G, Giovannini M
Scand J Gastroenterol 2011 Jul;46(7-8):1014-9. Epub 2011 Apr 15 doi: 10.3109/00365521.2011.571711. PMID: 21492053
Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases.
Relles D, Baek J, Witkiewicz A, Yeo CJ
J Gastrointest Surg 2010 Jun;14(6):1052-61. Epub 2010 Mar 19 doi: 10.1007/s11605-009-1123-0. PMID: 20300877
Natural killer cell activity in patients with neuroendocrine tumours of the gastrointestinal tract; relation with circulating gastrointestinal hormones.
Aparicio-Pagés MN, Verspaget HW, Peña AS, Jansen JB, Lamers CB
Neuropeptides 1991 Sep;20(1):1-7. doi: 10.1016/0143-4179(91)90033-f. PMID: 1791920

Diagnosis

Familial Duodenal Somatostatinomatosis Not Associated With a Known Genetic Syndrome.
Yu R, Rao J, Zhai J, Hecht JR, Pisegna JR
Pancreas 2022 Sep 1;51(8):1056-1060. doi: 10.1097/MPA.0000000000002126. PMID: 36607953
Endoscopic Ampullectomy of a Somatostatinoma.
Trindade AJ, Benias PC, Goldenberg S
Clin Gastroenterol Hepatol 2021 Mar;19(3):e24-e25. Epub 2020 Jan 15 doi: 10.1016/j.cgh.2020.01.010. PMID: 31954200
Neuroendocrine tumors: characterization with contrast-enhanced ultrasonography.
Dörffel Y, Wermke W
Ultraschall Med 2008 Oct;29(5):506-14. doi: 10.1055/s-2008-1027555. PMID: 19241507
Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen's disease. Case report and review of the literature.
Sakorafas GH, Giannopoulos GA, Parasi A, Konstantoudakis G, Tzanakis N, Stergiopoulos S, Peros G
JOP 2008 Sep 2;9(5):633-9. PMID: 18762695
Gut hormone secreting tumours.
Wood SM, Polak JM, Bloom SR
Scand J Gastroenterol Suppl 1983;82:165-79. PMID: 6314485

Therapy

Familial Duodenal Somatostatinomatosis Not Associated With a Known Genetic Syndrome.
Yu R, Rao J, Zhai J, Hecht JR, Pisegna JR
Pancreas 2022 Sep 1;51(8):1056-1060. doi: 10.1097/MPA.0000000000002126. PMID: 36607953
Intractable and dramatic diarrhea in liver transplantation recipient with vasoactive intestinal peptide-producing tumor after split liver transplantation: a case report.
Haiqing W, Jiayin Y, Jian Y, Lunan Y
Transplant Proc 2015 Jan-Feb;47(1):171-3. Epub 2015 Jan 14 doi: 10.1016/j.transproceed.2014.07.078. PMID: 25596962
Short-term results of 42 endoscopic ampullectomies: a single-center experience.
Jeanniard-Malet O, Caillol F, Pesenti C, Bories E, Monges G, Giovannini M
Scand J Gastroenterol 2011 Jul;46(7-8):1014-9. Epub 2011 Apr 15 doi: 10.3109/00365521.2011.571711. PMID: 21492053
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
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

LONG-TERM FOLLOW-UP OF DUODENAL PAPILLARY SOMATOSTATINOMA TREATED BY ENDOSCOPIC PAPILLECTOMY.
Ardengh JC, Ardengh AO, Almeida AF, Machado MA
Arq Gastroenterol 2021 Jul-Sep;58(3):411-412. doi: 10.1590/S0004-2803.202100000-71. PMID: 34705982
Synchronous endocrine tumors of small intestine: report of a case.
Sen N, Calli Demirkan N, Aksoy Altinboğa A, Bolat H, Erdem E
Turk J Gastroenterol 2008 Sep;19(3):193-6. PMID: 19115157
Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen's disease. Case report and review of the literature.
Sakorafas GH, Giannopoulos GA, Parasi A, Konstantoudakis G, Tzanakis N, Stergiopoulos S, Peros G
JOP 2008 Sep 2;9(5):633-9. PMID: 18762695
In vitro identification of vasoactive intestinal peptide receptors in human tumors: implications for tumor imaging.
Reubi JC
J Nucl Med 1995 Oct;36(10):1846-53. PMID: 7562054
Serum markers for pancreatic islet cell and intestinal carcinoid tumors: a comparison of neuron-specific enolase, beta-human chorionic gonadotropin and pancreatic polypeptide.
Prinz RA, Bermes EW Jr, Kimmel JR, Marangos PJ
Surgery 1983 Dec;94(6):1019-23. PMID: 6196846

Clinical prediction guides

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
Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease.
Usui M, Matsuda S, Suzuki H, Hirata K, Ogura Y, Shiraishi T
J Gastroenterol 2002;37(11):947-53. doi: 10.1007/s005350200159. PMID: 12483251
In vitro identification of vasoactive intestinal peptide receptors in human tumors: implications for tumor imaging.
Reubi JC
J Nucl Med 1995 Oct;36(10):1846-53. PMID: 7562054
Duodenal and ampullary carcinoid tumors. A report of 12 cases with pathological characteristics, polypeptide content and relation to the MEN I syndrome and von Recklinghausen's disease (neurofibromatosis).
Stamm B, Hedinger CE, Saremaslani P
Virchows Arch A Pathol Anat Histopathol 1986;408(5):475-89. doi: 10.1007/BF00705301. PMID: 2869609

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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