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Gastric neuroendocrine tumor G1

MedGen UID:
83885
•Concept ID:
C0349529
•
Neoplastic Process
Synonyms: Carcinoid tumor of stomach; Gastric Carcinoid Tumor; gastric carcinoid tumor; gastric carcinoid tumour; Gastric ECL Cell NET; gastric ECL cell NET; Gastric ECL Cell NET G1; gastric ECL cell NET G1; Gastric ECL Cell, Histamine-Producing NET; gastric ECL cell, histamine-producing NET; Gastric ECL-Cell Carcinoid Tumor; gastric ECL-cell carcinoid tumor; gastric ECL-cell carcinoid tumour; Gastric Enterochromaffin-Like Cell Carcinoid Tumor; gastric enterochromaffin-like cell carcinoid tumor; gastric enterochromaffin-like cell carcinoid tumour; Gastric Enterochromaffin-Like Cell Neuroendocrine Tumor; gastric enterochromaffin-like cell neuroendocrine tumor; gastric enterochromaffin-like cell neuroendocrine tumour; Gastric NET G1; gastric NET G1; Gastric Neuroendocrine Tumor G1; gastric neuroendocrine tumor G1; grade 1 neuroendocrine neoplasm of stomach; stomach carcinoid tumor; stomach carcinoid tumour; stomach NET G1; stomach neuroendocrine neoplasm G1; stomach neuroendocrine tumor, well differentiated, low grade
SNOMED CT: Carcinoid tumor of stomach (276808007)
 
Monarch Initiative: MONDO:0006227

Definition

A well differentiated, low grade neuroendocrine neoplasm (carcinoid tumor) that arises from the stomach. The vast majority of cases arise from the corpus-fundus region. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent. It may be associated with autoimmune chronic atrophic gastritis, multiple endocrine neoplasia type 1, or it may be sporadic. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGastric neuroendocrine tumor G1

Professional guidelines

PubMed

Systemic Therapy for Tumor Control in Metastatic Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline.
Del Rivero J, Perez K, Kennedy EB, Mittra ES, Vijayvergia N, Arshad J, Basu S, Chauhan A, Dasari AN, Bellizzi AM, Gangi A, Grady E, Howe JR, Ivanidze J, Lewis M, Mailman J, Raj N, Soares HP, Soulen MC, White SB, Chan JA, Kunz PL, Singh S, Halfdanarson TR, Strosberg JR, Bergsland EK
J Clin Oncol 2023 Nov 10;41(32):5049-5067. Epub 2023 Sep 29 doi: 10.1200/JCO.23.01529. PMID: 37774329
Management of gastric and duodenal neuroendocrine tumors.
Sato Y, Hashimoto S, Mizuno K, Takeuchi M, Terai S
World J Gastroenterol 2016 Aug 14;22(30):6817-28. doi: 10.3748/wjg.v22.i30.6817. PMID: 27570419Free PMC Article
Gastroenteropancreatic Well-Differentiated Grade 3 Neuroendocrine Tumors: Review and Position Statement.
Coriat R, Walter T, Terris B, Couvelard A, Ruszniewski P
Oncologist 2016 Oct;21(10):1191-1199. Epub 2016 Jul 8 doi: 10.1634/theoncologist.2015-0476. PMID: 27401895Free PMC Article

Recent clinical studies

Etiology

Ampullary Neuroendocrine Neoplasm: Clinicopathological Characteristics and Novel Endoscopic Entity.
Matsumoto K, Fujimori N, Hata Y, Minoda Y, Murakami M, Teramatsu K, Takamatsu Y, Takeno A, Oono T, Ihara E, Nakata K, Nakamura M, Yamamoto T, Koga Y, Oda Y, Ito T, Ogawa Y
Dig Dis 2023;41(2):316-324. Epub 2022 May 19 doi: 10.1159/000525013. PMID: 35588707
PRRT for higher-grade neuroendocrine neoplasms: What is still acceptable?
Kong G, Hicks RJ
Curr Opin Pharmacol 2022 Dec;67:102293. Epub 2022 Oct 1 doi: 10.1016/j.coph.2022.102293. PMID: 36195008
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Pediatric gastroenteropancreatic neuroendocrine tumor: A case report and review of the literature.
Gaiani F, de'Angelis N, Minelli R, Kayali S, Carra MC, de'Angelis GL
Medicine (Baltimore) 2019 Sep;98(37):e17154. doi: 10.1097/MD.0000000000017154. PMID: 31517859Free PMC Article
Tumour type and size are prognostic factors in gastric neuroendocrine neoplasia: A multicentre retrospective study.
Panzuto F, Campana D, Massironi S, Faggiano A, Rinzivillo M, Lamberti G, Sciola V, Lahner E, Manuzzi L, Colao A, Annibale B
Dig Liver Dis 2019 Oct;51(10):1456-1460. Epub 2019 Jun 5 doi: 10.1016/j.dld.2019.04.016. PMID: 31175013

Diagnosis

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1-G3.
Panzuto F, Ramage J, Pritchard DM, van Velthuysen MF, Schrader J, Begum N, Sundin A, Falconi M, O'Toole D
J Neuroendocrinol 2023 Aug;35(8):e13306. Epub 2023 Jul 4 doi: 10.1111/jne.13306. PMID: 37401795
Ampullary Neuroendocrine Neoplasm: Clinicopathological Characteristics and Novel Endoscopic Entity.
Matsumoto K, Fujimori N, Hata Y, Minoda Y, Murakami M, Teramatsu K, Takamatsu Y, Takeno A, Oono T, Ihara E, Nakata K, Nakamura M, Yamamoto T, Koga Y, Oda Y, Ito T, Ogawa Y
Dig Dis 2023;41(2):316-324. Epub 2022 May 19 doi: 10.1159/000525013. PMID: 35588707
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Recurrence and metastatic potential in Type 1 gastric neuroendocrine neoplasms.
Daskalakis K, Tsoli M, Karapanagioti A, Chrysochoou M, Thomas D, Sougioultzis S, Karoumpalis I, Kaltsas GA, Alexandraki KI
Clin Endocrinol (Oxf) 2019 Oct;91(4):534-543. Epub 2019 Jul 7 doi: 10.1111/cen.14055. PMID: 31254407
Management of gastric and duodenal neuroendocrine tumors.
Sato Y, Hashimoto S, Mizuno K, Takeuchi M, Terai S
World J Gastroenterol 2016 Aug 14;22(30):6817-28. doi: 10.3748/wjg.v22.i30.6817. PMID: 27570419Free PMC Article

Therapy

Systemic Therapy for Tumor Control in Metastatic Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline.
Del Rivero J, Perez K, Kennedy EB, Mittra ES, Vijayvergia N, Arshad J, Basu S, Chauhan A, Dasari AN, Bellizzi AM, Gangi A, Grady E, Howe JR, Ivanidze J, Lewis M, Mailman J, Raj N, Soares HP, Soulen MC, White SB, Chan JA, Kunz PL, Singh S, Halfdanarson TR, Strosberg JR, Bergsland EK
J Clin Oncol 2023 Nov 10;41(32):5049-5067. Epub 2023 Sep 29 doi: 10.1200/JCO.23.01529. PMID: 37774329
Role of immunotherapy in gastro-enteropancreatic neuroendocrine neoplasms (gep-nens): Current advances and future directions.
Fang L, Arvind D, Dowlati A, Mohamed A
J Neuroendocrinol 2021 Mar;33(3):e12943. doi: 10.1111/jne.12943. PMID: 33724586
BRAF(V600E) mutation: A promising target in colorectal neuroendocrine carcinoma.
Dizdar L, Werner TA, Drusenheimer JC, Möhlendick B, Raba K, Boeck I, Anlauf M, Schott M, Göring W, Esposito I, Stoecklein NH, Knoefel WT, Krieg A
Int J Cancer 2019 Mar 15;144(6):1379-1390. Epub 2018 Oct 30 doi: 10.1002/ijc.31828. PMID: 30144031
Inhibition of histone deacetylases in melanoma-a perspective from bench to bedside.
Hornig E, Heppt MV, Graf SA, Ruzicka T, Berking C
Exp Dermatol 2016 Nov;25(11):831-838. doi: 10.1111/exd.13089. PMID: 27792246
Biotherapies for GEP-NETs.
Öberg K
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):833-41. doi: 10.1016/j.bpg.2013.01.001. PMID: 23582922

Prognosis

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1-G3.
Panzuto F, Ramage J, Pritchard DM, van Velthuysen MF, Schrader J, Begum N, Sundin A, Falconi M, O'Toole D
J Neuroendocrinol 2023 Aug;35(8):e13306. Epub 2023 Jul 4 doi: 10.1111/jne.13306. PMID: 37401795
Ampullary Neuroendocrine Neoplasm: Clinicopathological Characteristics and Novel Endoscopic Entity.
Matsumoto K, Fujimori N, Hata Y, Minoda Y, Murakami M, Teramatsu K, Takamatsu Y, Takeno A, Oono T, Ihara E, Nakata K, Nakamura M, Yamamoto T, Koga Y, Oda Y, Ito T, Ogawa Y
Dig Dis 2023;41(2):316-324. Epub 2022 May 19 doi: 10.1159/000525013. PMID: 35588707
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Gastro-entero-pancreatic neuroendocrine neoplasia: The rules for non-operative management.
Panzuto F, Massironi S, Partelli S, Campana D, Rinzivillo M, Invernizzi P, Andreasi V, Lamberti G, Falconi M
Surg Oncol 2020 Dec;35:141-148. Epub 2020 Aug 20 doi: 10.1016/j.suronc.2020.08.015. PMID: 32877883
Recurrence and metastatic potential in Type 1 gastric neuroendocrine neoplasms.
Daskalakis K, Tsoli M, Karapanagioti A, Chrysochoou M, Thomas D, Sougioultzis S, Karoumpalis I, Kaltsas GA, Alexandraki KI
Clin Endocrinol (Oxf) 2019 Oct;91(4):534-543. Epub 2019 Jul 7 doi: 10.1111/cen.14055. PMID: 31254407

Clinical prediction guides

Systemic Therapy for Tumor Control in Metastatic Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline.
Del Rivero J, Perez K, Kennedy EB, Mittra ES, Vijayvergia N, Arshad J, Basu S, Chauhan A, Dasari AN, Bellizzi AM, Gangi A, Grady E, Howe JR, Ivanidze J, Lewis M, Mailman J, Raj N, Soares HP, Soulen MC, White SB, Chan JA, Kunz PL, Singh S, Halfdanarson TR, Strosberg JR, Bergsland EK
J Clin Oncol 2023 Nov 10;41(32):5049-5067. Epub 2023 Sep 29 doi: 10.1200/JCO.23.01529. PMID: 37774329
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Expression of LAT1 and 4F2hc in Gastroenteropancreatic Neuroendocrine Neoplasms.
Horita Y, Kaira K, Kawasaki T, Mihara Y, Sakuramoto S, Yamaguchi S, Okamoto K, Ryozawa S, Kanai Y, Yasuda M, Hamaguchi T
In Vivo 2021 Jul-Aug;35(4):2425-2432. doi: 10.21873/invivo.12520. PMID: 34182526Free PMC Article
Recurrence and metastatic potential in Type 1 gastric neuroendocrine neoplasms.
Daskalakis K, Tsoli M, Karapanagioti A, Chrysochoou M, Thomas D, Sougioultzis S, Karoumpalis I, Kaltsas GA, Alexandraki KI
Clin Endocrinol (Oxf) 2019 Oct;91(4):534-543. Epub 2019 Jul 7 doi: 10.1111/cen.14055. PMID: 31254407
Microenvironment and tumor inflammatory features improve prognostic prediction in gastro-entero-pancreatic neuroendocrine neoplasms.
Milione M, Miceli R, Barretta F, Pellegrinelli A, Spaggiari P, Tagliabue G, Centonze G, Paolino C, Mangogna A, Kankava K, Pusceddu S, Giacomelli L, Corti A, Cotsoglou C, Mazzaferro V, Sozzi G, de Braud F, Pruneri G, Anichini A
J Pathol Clin Res 2019 Oct;5(4):217-226. Epub 2019 Jul 9 doi: 10.1002/cjp2.135. PMID: 31136102Free PMC Article

Recent systematic reviews

Neuroendocrine neoplasms of gastrointestinal tract and secondary primary synchronous tumors: A systematic review of case reports. Casualty or causality?
Parra-Medina R, Moreno-Lucero P, Jimenez-Moreno J, Parra-Morales AM, Romero-Rojas A
PLoS One 2019;14(5):e0216647. Epub 2019 May 14 doi: 10.1371/journal.pone.0216647. PMID: 31086397Free PMC Article

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