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Malignant Pancreatic Gastrinoma

MedGen UID:
406818
•Concept ID:
C1881585
•
Neoplastic Process
Synonyms: Malignant G cell neoplasm of pancreas; Malignant gastrinoma of pancreas; Pancreatic G-Cell Carcinoma
SNOMED CT: Malignant G cell neoplasm of pancreas (1187519007); Malignant gastrinoma of pancreas (1187519007)

Definition

A gastrin-producing neuroendocrine tumor arising from the pancreas. It is characterized by inappropriate secretion of gastrin and associated with Zollinger Ellison syndrome. It displays vascular invasion and metastasizes to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Pancreatic Gastrinoma

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
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
Pancreatic neuroendocrine tumours.
O'Grady HL, Conlon KC
Eur J Surg Oncol 2008 Mar;34(3):324-32. Epub 2007 Oct 29 doi: 10.1016/j.ejso.2007.07.209. PMID: 17967523

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
Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
Ito T, Jensen RT
Expert Opin Pharmacother 2021 Apr;22(6):685-693. Epub 2020 Nov 11 doi: 10.1080/14656566.2020.1845651. PMID: 33131345Free 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

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
Multiple endocrine neoplasms.
Falchetti A, Marini F, Luzi E, Tonelli F, Brandi ML
Best Pract Res Clin Rheumatol 2008 Mar;22(1):149-63. doi: 10.1016/j.berh.2007.11.010. PMID: 18328987
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
Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances.
Guilmette JM, Nosé V
Adv Anat Pathol 2019 Jan;26(1):13-30. doi: 10.1097/PAP.0000000000000201. PMID: 29912000
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
Italian Association of Clinical Endocrinologists (AME) and Italian AACE Chapter Position Statement for Clinical Practice: Assessment of Response to Treatment and Follow-Up in Gastroenteropancreatic Neuroendocrine Neoplasms.
Grimaldi F, Fazio N, Attanasio R, Frasoldati A, Papini E, Cremonini N, Davi MV, Funicelli L, Massironi S, Spada F, Toscano V, Versari A, Zini M, Falconi M, Oberg K
Endocr Metab Immune Disord Drug Targets 2018;18(5):419-449. doi: 10.2174/1871530318666171213145803. PMID: 29237387
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
Proteomics of Pancreatic Neuroendocrine Tumors: A Systematic Review.
Maiorano BA, Schinzari G, Chiloiro S, Visconti F, Milardi D, Bianchi A
Protein Pept Lett 2020;27(12):1276-1287. doi: 10.2174/0929866527666200504122116. PMID: 31804164
Liver transplantation for hepatic metastases of neuroendocrine pancreatic tumors: a survival-based analysis.
Máthé Z, Tagkalos E, Paul A, Molmenti EP, Kóbori L, Fouzas I, Beckebaum S, Sotiropoulos GC
Transplantation 2011 Mar 15;91(5):575-82. doi: 10.1097/TP.0b013e3182081312. PMID: 21200365

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