U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Duodenal gastrin-producing neuroendocrine tumor

MedGen UID:
272550
•Concept ID:
C1333321
•
Neoplastic Process
Synonyms: Duodenal G-Cell Gastrin Producing Tumor; duodenal G-cell gastrin producing tumor; duodenal G-cell gastrin producing tumour; Duodenal Gastrin-Producing NET; duodenal gastrin-producing NET; Duodenal Gastrin-Producing Neuroendocrine Tumor; duodenal gastrin-producing neuroendocrine tumor; Duodenal Gastrinoma; duodenal gastrinoma; duodenum gastrin-producing neuroendocrine tumor; duodenum gastrin-producing neuroendocrine tumour; Gastrin Producing Tumor of Duodenum; gastrin producing tumor of duodenum; Gastrin Producing Tumor of the Duodenum; gastrin producing tumor of the duodenum; gastrin producing tumour of duodenum; gastrin producing tumour of the duodenum; gastrin-producing neuroendocrine tumor of duodenum; gastrin-producing neuroendocrine tumour of duodenum; Gastrinoma of duodenum; Gastrinoma of Duodenum; gastrinoma of duodenum; Gastrinoma of the Duodenum; gastrinoma of the duodenum; malignant duodenal gastrinoma
SNOMED CT: Gastrinoma of duodenum (879992009)
 
Monarch Initiative: MONDO:0004411

Definition

A gastrin-producing neuroendocrine tumor that arises from the duodenum. 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]

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

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
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
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

Contemporary surgical management of the Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1.
Albers MB, Manoharan J, Bartsch DK
Best Pract Res Clin Endocrinol Metab 2019 Oct;33(5):101318. Epub 2019 Aug 31 doi: 10.1016/j.beem.2019.101318. PMID: 31521501
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
Medical treatment of gastrinomas.
Auernhammer CJ, Göke B
Wien Klin Wochenschr 2007;119(19-20):609-15. doi: 10.1007/s00508-007-0877-1. PMID: 17985097

Prognosis

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
Duodenal neuroendocrine neoplasms: a still poorly recognized clinical entity.
Rossi RE, Rausa E, Cavalcoli F, Conte D, Massironi S
Scand J Gastroenterol 2018 Jun-Jul;53(7):835-842. Epub 2018 May 4 doi: 10.1080/00365521.2018.1468479. PMID: 29726295
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
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

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
Development and Validation of Prognostic Nomograms for Patients with Duodenal Neuroendocrine Neoplasms.
Sun S, Wang W, He C
Med Sci Monit 2020 Jun 21;26:e922613. doi: 10.12659/MSM.922613. PMID: 32564052Free 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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...