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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; Pancreatic Gastrinoma
 
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

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.
Damaskos C, Dimitroulis D, Garmpi A, Antoniou EA, Kouraklis G, Psilopatis I, Mavri M, Diamantis E, Marinos G, Kyriakos G, Farmaki P, Patsouras A, Kontzoglou K, Garmpis N
In Vivo 2023 Nov-Dec;37(6):2402-2408. doi: 10.21873/invivo.13345. PMID: 37905620Free 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
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.
Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
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

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
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
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
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

Therapy

Management of Functional Pancreatic Neuroendocrine Neoplasms.
Magi L, Marasco M, Rinzivillo M, Faggiano A, Panzuto F
Curr Treat Options Oncol 2023 Jul;24(7):725-741. Epub 2023 Apr 27 doi: 10.1007/s11864-023-01085-0. PMID: 37103745Free 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
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
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

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
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
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
A clinical analysis on functioning pancreatic neuroendocrine tumors (focusing on VIPomas): a single-center experience.
Murakami M, Fujimori N, Matsumoto K, Ohno A, Teramatsu K, Takamatsu Y, Takeno A, Ueda K, Oono T, Ito T, Ogawa Y
Endocr J 2022 Oct 28;69(10):1201-1209. Epub 2022 May 27 doi: 10.1507/endocrj.EJ22-0111. PMID: 35644576
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

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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