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Pancreatic gastrin-producing neuroendocrine tumor

MedGen UID:
235080
•Concept ID:
C1368066
•
Neoplastic Process
Synonyms: gastrin-producing neuroendocrine tumor of pancreas; gastrin-producing neuroendocrine tumour of pancreas; islet cell tumor, ulcerogenic; pancreas gastrin-producing neuroendocrine tumor; pancreas gastrin-producing neuroendocrine tumour; pancreatic G-cell tumor; pancreatic G-cell tumour; Pancreatic Gastrin Producing NET; pancreatic gastrin producing NET; Pancreatic Gastrin Producing Tumor; pancreatic gastrin producing tumor; pancreatic gastrin producing tumour; Pancreatic Gastrin-Producing Neuroendocrine Tumor; pancreatic gastrin-producing neuroendocrine tumor
 
Monarch Initiative: MONDO:0003525

Definition

A usually malignant gastrin-producing neuroendocrine tumor arising from the pancreas. It may or may not be associated with inappropriate secretion of gastrin and an associated clinical syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic gastrin-producing neuroendocrine tumor

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
An Organoid Biobank of Neuroendocrine Neoplasms Enables Genotype-Phenotype Mapping.
Kawasaki K, Toshimitsu K, Matano M, Fujita M, Fujii M, Togasaki K, Ebisudani T, Shimokawa M, Takano A, Takahashi S, Ohta Y, Nanki K, Igarashi R, Ishimaru K, Ishida H, Sukawa Y, Sugimoto S, Saito Y, Maejima K, Sasagawa S, Lee H, Kim HG, Ha K, Hamamoto J, Fukunaga K, Maekawa A, Tanabe M, Ishihara S, Hamamoto Y, Yasuda H, Sekine S, Kudo A, Kitagawa Y, Kanai T, Nakagawa H, Sato T
Cell 2020 Nov 25;183(5):1420-1435.e21. Epub 2020 Nov 6 doi: 10.1016/j.cell.2020.10.023. PMID: 33159857
Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.
Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article

Recent clinical studies

Etiology

Management of Gastric Neuroendocrine Tumors: A Review.
Sok C, Ajay PS, Tsagkalidis V, Kooby DA, Shah MM
Ann Surg Oncol 2024 Mar;31(3):1509-1518. Epub 2023 Dec 7 doi: 10.1245/s10434-023-14712-9. PMID: 38062290Free 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
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free 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
Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266

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
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Imaging of pancreatic neuroendocrine tumors: recent advances, current status, and controversies.
Lee L, Ito T, Jensen RT
Expert Rev Anticancer Ther 2018 Sep;18(9):837-860. Epub 2018 Jul 17 doi: 10.1080/14737140.2018.1496822. PMID: 29973077Free PMC Article
Zollinger-Ellison syndrome: recent advances and controversies.
Ito T, Igarashi H, Jensen RT
Curr Opin Gastroenterol 2013 Nov;29(6):650-61. doi: 10.1097/MOG.0b013e328365efb1. PMID: 24100728Free PMC Article
Pharmacotherapy of Zollinger-Ellison syndrome.
Ito T, Igarashi H, Uehara H, Jensen RT
Expert Opin Pharmacother 2013 Feb;14(3):307-21. Epub 2013 Jan 30 doi: 10.1517/14656566.2013.767332. PMID: 23363383Free PMC Article

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
Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.
Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article
Pharmacotherapy of Zollinger-Ellison syndrome.
Ito T, Igarashi H, Uehara H, Jensen RT
Expert Opin Pharmacother 2013 Feb;14(3):307-21. Epub 2013 Jan 30 doi: 10.1517/14656566.2013.767332. PMID: 23363383Free PMC Article
Pancreatic neuroendocrine tumours.
Alexakis N, Neoptolemos JP
Best Pract Res Clin Gastroenterol 2008;22(1):183-205. doi: 10.1016/j.bpg.2007.10.008. PMID: 18206821
Gastrinoma.
Li ML, Norton JA
Curr Treat Options Oncol 2001 Aug;2(4):337-46. doi: 10.1007/s11864-001-0027-3. PMID: 12057114

Prognosis

Management of Gastric Neuroendocrine Tumors: A Review.
Sok C, Ajay PS, Tsagkalidis V, Kooby DA, Shah MM
Ann Surg Oncol 2024 Mar;31(3):1509-1518. Epub 2023 Dec 7 doi: 10.1245/s10434-023-14712-9. PMID: 38062290Free PMC Article
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Imaging of pancreatic neuroendocrine tumors: recent advances, current status, and controversies.
Lee L, Ito T, Jensen RT
Expert Rev Anticancer Ther 2018 Sep;18(9):837-860. Epub 2018 Jul 17 doi: 10.1080/14737140.2018.1496822. PMID: 29973077Free PMC Article
Zollinger-Ellison syndrome: recent advances and controversies.
Ito T, Igarashi H, Jensen RT
Curr Opin Gastroenterol 2013 Nov;29(6):650-61. doi: 10.1097/MOG.0b013e328365efb1. PMID: 24100728Free 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
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Sporadic pancreatic neuroendocrine tumor: Surgery of the primary tumor.
Deguelte S, de Mestier L, Hentic O, Cros J, Lebtahi R, Hammel P, Kianmanesh R
J Visc Surg 2018 Dec;155(6):483-492. Epub 2018 Nov 15 doi: 10.1016/j.jviscsurg.2018.08.010. PMID: 30448206
Imaging of pancreatic neoplasms.
Balachandran A, Bhosale PR, Charnsangavej C, Tamm EP
Surg Oncol Clin N Am 2014 Oct;23(4):751-88. doi: 10.1016/j.soc.2014.07.002. PMID: 25246049
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

Recent systematic reviews

Radioguided Surgery for Gastroenteropancreatic Neuroendocrine Tumours: a Systematic Literature Review.
Cockburn KC, Toumi Z, Mackie A, Julyan P
J Gastrointest Surg 2021 Dec;25(12):3244-3257. Epub 2021 Sep 10 doi: 10.1007/s11605-021-05115-w. PMID: 34506015Free PMC Article

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