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Duodenal gastrin-producing neuroendocrine tumor

MedGen UID:
272550
•Concept ID:
C1333321
•
Neoplastic Process
Synonym: Duodenal gastrinoma
SNOMED CT: Gastrinoma of duodenum (879992009)
 
HPO: HP:0034953
Monarch Initiative: MONDO:0004411

Definition

A gastrinoma, a neuroendocrine tumors characterized by the secretion of gastrin with resultant excessive gastric acid production, that is localized to the duodenum. [from HPO]

Professional guidelines

PubMed

Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies.
Rossi RE, Elvevi A, Citterio D, Coppa J, Invernizzi P, Mazzaferro V, Massironi S
World J Gastroenterol 2021 Sep 21;27(35):5890-5907. doi: 10.3748/wjg.v27.i35.5890. PMID: 34629807Free PMC Article
Diagnosis and management of Zollinger-Ellison syndrome in 2018.
De Angelis C, Cortegoso Valdivia P, Venezia L, Bruno M, Pellicano R
Minerva Endocrinol 2018 Jun;43(2):212-220. Epub 2017 Sep 25 doi: 10.23736/S0391-1977.17.02745-6. PMID: 28949124
Hypergastrinemia--diagnosis and treatment.
Niederle B
Wien Klin Wochenschr 2007;119(19-20):561-3. doi: 10.1007/s00508-007-0897-x. PMID: 17985087

Recent clinical studies

Etiology

Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies.
Rossi RE, Elvevi A, Citterio D, Coppa J, Invernizzi P, Mazzaferro V, Massironi S
World J Gastroenterol 2021 Sep 21;27(35):5890-5907. doi: 10.3748/wjg.v27.i35.5890. PMID: 34629807Free PMC Article
Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.
Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025Free PMC Article
Diagnosis and management of Zollinger-Ellison syndrome in 2018.
De Angelis C, Cortegoso Valdivia P, Venezia L, Bruno M, Pellicano R
Minerva Endocrinol 2018 Jun;43(2):212-220. Epub 2017 Sep 25 doi: 10.23736/S0391-1977.17.02745-6. PMID: 28949124
Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome.
Norton JA, Fraker DL, Alexander HR, Jensen RT
Ann Surg 2012 Sep;256(3):509-17. doi: 10.1097/SLA.0b013e318265f08d. PMID: 22868363Free PMC Article
Peptic ulcer pathophysiology.
Mertz HR, Walsh JH
Med Clin North Am 1991 Jul;75(4):799-814. doi: 10.1016/s0025-7125(16)30412-6. PMID: 2072787

Diagnosis

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
All you need to know about gastrinoma today | Gastrinoma and Zollinger-Ellison syndrome: A thorough update.
Chatzipanagiotou O, Schizas D, Vailas M, Tsoli M, Sakarellos P, Sotiropoulou M, Papalambros A, Felekouras E
J Neuroendocrinol 2023 Apr;35(4):e13267. Epub 2023 Apr 11 doi: 10.1111/jne.13267. PMID: 37042078
Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies.
Rossi RE, Elvevi A, Citterio D, Coppa J, Invernizzi P, Mazzaferro V, Massironi S
World J Gastroenterol 2021 Sep 21;27(35):5890-5907. doi: 10.3748/wjg.v27.i35.5890. PMID: 34629807Free PMC Article
Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.
Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025Free 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

Therapy

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.
Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025Free PMC Article
Current management of the Zollinger-Ellison syndrome.
Krampitz GW, Norton JA
Adv Surg 2013;47:59-79. doi: 10.1016/j.yasu.2013.02.004. PMID: 24298844
Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome.
Norton JA, Fraker DL, Alexander HR, Jensen RT
Ann Surg 2012 Sep;256(3):509-17. doi: 10.1097/SLA.0b013e318265f08d. PMID: 22868363Free PMC Article
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
Histamine and the gut.
Hirschowitz BI
N Engl Reg Allergy Proc 1985 Winter;6(1):21-7. doi: 10.2500/108854185779048942. PMID: 3916579

Prognosis

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.
Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025Free PMC Article
Diagnosis and management of Zollinger-Ellison syndrome in 2018.
De Angelis C, Cortegoso Valdivia P, Venezia L, Bruno M, Pellicano R
Minerva Endocrinol 2018 Jun;43(2):212-220. Epub 2017 Sep 25 doi: 10.23736/S0391-1977.17.02745-6. PMID: 28949124
Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome.
Norton JA, Fraker DL, Alexander HR, Jensen RT
Ann Surg 2012 Sep;256(3):509-17. doi: 10.1097/SLA.0b013e318265f08d. PMID: 22868363Free 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
Gastrinoma (duodenal and pancreatic).
Jensen RT, Niederle B, Mitry E, Ramage JK, Steinmuller T, Lewington V, Scarpa A, Sundin A, Perren A, Gross D, O'Connor JM, Pauwels S, Kloppel G; Frascati Consensus Conference; European Neuroendocrine Tumor Society
Neuroendocrinology 2006;84(3):173-82. Epub 2007 Feb 20 doi: 10.1159/000098009. PMID: 17312377

Clinical prediction guides

How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?
Imamura M, Komoto I, Taki Y
Surg Today 2023 Dec;53(12):1325-1334. Epub 2022 Dec 7 doi: 10.1007/s00595-022-02627-z. PMID: 36473964Free PMC Article
Sporadic, non-functional, gastrin-producing duodenal neuroendocrine tumors: A retrospective study of an infrequent disease.
de Jorge Huerta L, Solares Fernández I, Sánchez-Moreno B, Males Maldonado D, de Ibarrola Andrés C, Díaz-Simón R
J Dig Dis 2022 Aug;23(8-9):455-461. Epub 2022 Nov 10 doi: 10.1111/1751-2980.13129. PMID: 36168962Free PMC Article
Neuroendocrine pathology of the stomach: the Parma contribution.
Bordi C
Endocr Pathol 2014 Jun;25(2):171-80. doi: 10.1007/s12022-014-9315-x. PMID: 24782101
Primary nodal gastrinomas.
Herrmann ME, Ciesla MC, Chejfec G, DeJong SA, Yong SL
Arch Pathol Lab Med 2000 Jun;124(6):832-5. doi: 10.5858/2000-124-0832-PNG. PMID: 10835515
Pathobiology and management of hypergastrinemia and the Zollinger-Ellison syndrome.
Hirschowitz BI
Yale J Biol Med 1992 Nov-Dec;65(6):659-76; discussion 689-92. PMID: 1341070Free PMC Article

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