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Ampulla of Vater Somatostatin-Producing Neuroendocrine Tumor

MedGen UID:
474117
•Concept ID:
C3272484
•
Neoplastic Process
Synonyms: Ampulla of Vater Somatostatin Producing NET; Ampulla of Vater Somatostatin Producing Neuroendocrine Tumor; Somatostatin Producing Neuroendocrine Tumor of the Ampullary Region

Definition

A well differentiated neuroendocrine tumor that arises from the ampulla of Vater and the periampullary region. It produces somatostatin. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpulla of Vater Somatostatin-Producing Neuroendocrine Tumor

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
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
Somatostatinoma of the ampulla of vater in celiac sprue.
Frick EJ Jr, Kralstein JR, Scarlato M, Hoover HC Jr
J Gastrointest Surg 2000 Jul-Aug;4(4):388-91. doi: 10.1016/s1091-255x(00)80017-7. PMID: 11058857
Carcinoid somatostatinoma of the papilla of Vater: a case report.
Yoshida T, Matsumoto T, Morii Y, Shibata K, Fujii K, Aramaki M, Kitano S
Hepatogastroenterology 1998 Mar-Apr;45(20):451-3. PMID: 9638424

Diagnosis

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 (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
Pancreatic and peripancreatic somatostatinomas.
Williamson JM, Thorn CC, Spalding D, Williamson RC
Ann R Coll Surg Engl 2011 Jul;93(5):356-60. doi: 10.1308/003588411X582681. PMID: 21943457Free PMC Article
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
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

Therapy

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
Somatostatinoma: a case report and review of the literature.
Harris GJ, Tio F, Cruz AB Jr
J Surg Oncol 1987 Sep;36(1):8-16. doi: 10.1002/jso.2930360104. PMID: 3041116

Prognosis

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
Duodenal somatostatinoma of the ampulla of vater diagnosed by endoscopic fine needle aspiration biopsy: a case report.
Guo M, Lemos LB, Bigler S, Baliga M
Acta Cytol 2001 Jul-Aug;45(4):622-6. doi: 10.1159/000327876. PMID: 11480730
Somatostatinoma of the ampulla of vater in celiac sprue.
Frick EJ Jr, Kralstein JR, Scarlato M, Hoover HC Jr
J Gastrointest Surg 2000 Jul-Aug;4(4):388-91. doi: 10.1016/s1091-255x(00)80017-7. PMID: 11058857
Carcinoid somatostatinoma of the papilla of Vater: a case report.
Yoshida T, Matsumoto T, Morii Y, Shibata K, Fujii K, Aramaki M, Kitano S
Hepatogastroenterology 1998 Mar-Apr;45(20):451-3. PMID: 9638424

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
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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