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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
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
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
The current management of pancreatic neuroendocrine tumors.
Ellison TA, Edil BH
Adv Surg 2012;46:283-96. doi: 10.1016/j.yasu.2012.04.002. PMID: 22873046
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

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
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
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.
Chambers AJ, Pasieka JL
Cancer Treat Res 2010;153:213-33. doi: 10.1007/978-1-4419-0857-5_12. PMID: 19957227
Hypergastrinemia--diagnosis and treatment.
Niederle B
Wien Klin Wochenschr 2007;119(19-20):561-3. doi: 10.1007/s00508-007-0897-x. PMID: 17985087

Therapy

Sequential therapies in gastric gastrinoma.
Rosiek V
Endokrynol Pol 2022;73(1):171-172. doi: 10.5603/EP.a2021.0101. PMID: 35381105
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
Minimally Invasive Techniques for Resection of Pancreatic Neuroendocrine Tumors.
Fernandez Ranvier GG, Shouhed D, Inabnet WB 3rd
Surg Oncol Clin N Am 2016 Jan;25(1):195-215. Epub 2015 Oct 31 doi: 10.1016/j.soc.2015.08.009. PMID: 26610782
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
Octreotide.
Lancet 1989 Sep 2;2(8662):541-2. PMID: 2570240

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
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
Imaging of secretory tumors of the gastrointestinal tract.
ElGuindy YM, Javadi S, Menias CO, Jensen CT, Elsamaloty H, Elsayes KM
Abdom Radiol (NY) 2017 Apr;42(4):1113-1131. doi: 10.1007/s00261-016-0976-4. PMID: 27878636
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
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
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
Arterial stimulation and venous sampling for glucagonomas of the pancreas.
Eguchi H, Tanemura M, Marubashi S, Kobayashi S, Wada H, Okita K, Iwahashi H, Imagawa A, Shimomura I, Osuga K, Umeshita K, Wakasa K, Mori M, Doki Y, Nagano H
Hepatogastroenterology 2012 Jan-Feb;59(113):276-9. doi: 10.5754/hge11392. PMID: 21940356
Treatment with (90)Y- and (177)Lu-DOTATOC in patients with metastatic neuroendocrine tumors.
Frilling A, Weber F, Saner F, Bockisch A, Hofmann M, Mueller-Brand J, Broelsch CE
Surgery 2006 Dec;140(6):968-76; discussion 976-7. doi: 10.1016/j.surg.2006.07.030. PMID: 17188146
Unusual features of multiple endocrine neoplasia.
Frilling A, Becker H, Roeher HD
Henry Ford Hosp Med J 1992;40(3-4):253-5. PMID: 1362416

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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