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Digestive system neuroendocrine tumor, grade 1/2

MedGen UID:
443945
•Concept ID:
C2930967
•
Neoplastic Process
Synonyms: Gastro-enteropancreatic neuroendocrine tumor; Gastroenteropancreatic neuroendocrine tumor; GEP neuroendocrine tumor
 
Monarch Initiative: MONDO:0000386

Definition

A well-differentiated neuroendocrine neoplasm of low, intermediate, or high grade arising from the digestive system. It is characterized by the presence of cells with features similar to those of the normal endocrine cells of the digestive system. The neoplastic cells express immunohistochemical evidence of neuroendocrine differentiation and hormones. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDigestive system neuroendocrine tumor, grade 1/2

Professional guidelines

PubMed

AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review.
Shah SC, Piazuelo MB, Kuipers EJ, Li D
Gastroenterology 2021 Oct;161(4):1325-1332.e7. Epub 2021 Aug 26 doi: 10.1053/j.gastro.2021.06.078. PMID: 34454714Free PMC Article

Recent clinical studies

Etiology

Well-differentiated neuroendocrine tumors of the appendix: Diagnosis, differentials, and disease progression.
Navale P, Gonzalez RS
Semin Diagn Pathol 2024 Sep;41(5):236-242. Epub 2024 Aug 14 doi: 10.1053/j.semdp.2024.08.001. PMID: 39168792
Effectiveness of Etoposide and Cisplatin vs Irinotecan and Cisplatin Therapy for Patients With Advanced Neuroendocrine Carcinoma of the Digestive System: The TOPIC-NEC Phase 3 Randomized Clinical Trial.
Morizane C, Machida N, Honma Y, Okusaka T, Boku N, Kato K, Nomura S, Hiraoka N, Sekine S, Taniguchi H, Okano N, Yamaguchi K, Sato T, Ikeda M, Mizuno N, Ozaka M, Kataoka T, Ueno M, Kitagawa Y, Terashima M, Furuse J; Japan Clinical Oncology Group (JCOG)
JAMA Oncol 2022 Oct 1;8(10):1447-1455. doi: 10.1001/jamaoncol.2022.3395. PMID: 35980649Free PMC Article
AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review.
Shah SC, Piazuelo MB, Kuipers EJ, Li D
Gastroenterology 2021 Oct;161(4):1325-1332.e7. Epub 2021 Aug 26 doi: 10.1053/j.gastro.2021.06.078. PMID: 34454714Free PMC Article
Comparison between neuroendocrine carcinomas and well-differentiated neuroendocrine tumors of the pancreas using dynamic enhanced CT.
Park HJ, Kim HJ, Kim KW, Kim SY, Choi SH, You MW, Hwang HS, Hong SM
Eur Radiol 2020 Sep;30(9):4772-4782. Epub 2020 Apr 28 doi: 10.1007/s00330-020-06867-w. PMID: 32346794
Duodenal neuroendocrine tumors: Impact of tumor size and total number of lymph nodes examined.
Zhang XF, Wu XN, Tsilimigras DI, Poultsides G, Rocha F, Abbott DE, Fields R, Idrees K, Cho C, Maithel SK, Pawlik TM; other members of the US Neuroendocrine Tumor Study Group
J Surg Oncol 2019 Dec;120(8):1302-1310. Epub 2019 Nov 3 doi: 10.1002/jso.25753. PMID: 31680243Free PMC Article

Diagnosis

Well-differentiated neuroendocrine tumors of the appendix: Diagnosis, differentials, and disease progression.
Navale P, Gonzalez RS
Semin Diagn Pathol 2024 Sep;41(5):236-242. Epub 2024 Aug 14 doi: 10.1053/j.semdp.2024.08.001. PMID: 39168792
Pancreatic Cancer Screening for At-Risk Individuals (Pancreas Scan Study): Yield, Harms, and Outcomes From a Prospective Multicenter Study.
Shah I, Silva-Santisteban A, Germansky KA, Trindade A, Raphael KL, Kushnir V, Pawa R, Mishra G, Anastasiou J, Inamdar S, Tharian B, Bilal M, Sawhney MS
Am J Gastroenterol 2023 Sep 1;118(9):1664-1670. Epub 2023 May 4 doi: 10.14309/ajg.0000000000002314. PMID: 37141538
AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review.
Shah SC, Piazuelo MB, Kuipers EJ, Li D
Gastroenterology 2021 Oct;161(4):1325-1332.e7. Epub 2021 Aug 26 doi: 10.1053/j.gastro.2021.06.078. PMID: 34454714Free PMC Article
Recurrence and Survival of Neuroendocrine Neoplasms of the Rectum: Single-Center Experience.
Storino A, Wong D, Ore AS, Gaytan-Fuentes IA, Fabrizio A, Cataldo T, Messaris E
J Gastrointest Surg 2021 Sep;25(9):2398-2400. Epub 2020 Dec 2 doi: 10.1007/s11605-020-04854-6. PMID: 33269457
Comparison between neuroendocrine carcinomas and well-differentiated neuroendocrine tumors of the pancreas using dynamic enhanced CT.
Park HJ, Kim HJ, Kim KW, Kim SY, Choi SH, You MW, Hwang HS, Hong SM
Eur Radiol 2020 Sep;30(9):4772-4782. Epub 2020 Apr 28 doi: 10.1007/s00330-020-06867-w. PMID: 32346794

Therapy

Safety and efficacy of the FLOT regimen in the Polish population - an analysis of the prospective trial.
Marcisz-Grzanka K, Winiarek M, Pałucki J, Wieszczy P, Olesiński T, Jóźwiak M, Samsel R, Sułkowska U, Kolasińska-Ćwikła A, Wyrwicz LS
Neoplasma 2022 Dec;69(6):1445-1450. Epub 2022 Nov 11 doi: 10.4149/neo_2022_220720N734. PMID: 36353936
Effectiveness of Etoposide and Cisplatin vs Irinotecan and Cisplatin Therapy for Patients With Advanced Neuroendocrine Carcinoma of the Digestive System: The TOPIC-NEC Phase 3 Randomized Clinical Trial.
Morizane C, Machida N, Honma Y, Okusaka T, Boku N, Kato K, Nomura S, Hiraoka N, Sekine S, Taniguchi H, Okano N, Yamaguchi K, Sato T, Ikeda M, Mizuno N, Ozaka M, Kataoka T, Ueno M, Kitagawa Y, Terashima M, Furuse J; Japan Clinical Oncology Group (JCOG)
JAMA Oncol 2022 Oct 1;8(10):1447-1455. doi: 10.1001/jamaoncol.2022.3395. PMID: 35980649Free PMC Article
Prognostic factors for relapse in resected gastroenteropancreatic neuroendocrine neoplasms: A systematic review and meta-analysis.
Broadbent R, Wheatley R, Stajer S, Jacobs T, Lamarca A, Hubner RA, Valle JW, Amir E, McNamara MG
Cancer Treat Rev 2021 Dec;101:102299. Epub 2021 Oct 11 doi: 10.1016/j.ctrv.2021.102299. PMID: 34662810
Renal function in patients receiving streptozocin for locally advanced or metastatic digestive neuroendocrine tumours: results of the Streptotox-FFCD 0906 study.
Legoux JL, Lombard-Bohas C, Brixi H, Le Malicot K, Lecomte T, Dahan L, Ruszniewski P, Mahamat-Abakar A, Etienne PL, Caroli-Bosc FX, Dominguez S, Paule B, Terrebonne E, Michel P, Lepage C, Choukroun G; for Streptotox-FFCD0906 investigators
Clin Res Hepatol Gastroenterol 2021 Sep;45(5):101572. Epub 2021 Mar 19 doi: 10.1016/j.clinre.2020.10.014. PMID: 33751987
Digestive Neuroendocrine Tumor Distribution and Characteristics According to the 2010 WHO Classification: a Single Institution Experience in Lebanon.
Kourie HR, Ghorra C, Rassy M, Kesserouani C, Kattan J
Asian Pac J Cancer Prev 2016;17(5):2679-81. PMID: 27268650

Prognosis

Neuroendocrine neoplasms of the gallbladder: early detection and surgery is key to improved outcome.
Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review.
Shah SC, Piazuelo MB, Kuipers EJ, Li D
Gastroenterology 2021 Oct;161(4):1325-1332.e7. Epub 2021 Aug 26 doi: 10.1053/j.gastro.2021.06.078. PMID: 34454714Free PMC Article
Candidate protein biomarkers in pancreatic neuroendocrine neoplasms grade 3.
Ali AS, Perren A, Lindskog C, Welin S, Sorbye H, Grönberg M, Janson ET
Sci Rep 2020 Jun 30;10(1):10639. doi: 10.1038/s41598-020-67670-7. PMID: 32606315Free PMC Article
Duodenal neuroendocrine tumors: Impact of tumor size and total number of lymph nodes examined.
Zhang XF, Wu XN, Tsilimigras DI, Poultsides G, Rocha F, Abbott DE, Fields R, Idrees K, Cho C, Maithel SK, Pawlik TM; other members of the US Neuroendocrine Tumor Study Group
J Surg Oncol 2019 Dec;120(8):1302-1310. Epub 2019 Nov 3 doi: 10.1002/jso.25753. PMID: 31680243Free PMC Article
Digestive Neuroendocrine Tumor Distribution and Characteristics According to the 2010 WHO Classification: a Single Institution Experience in Lebanon.
Kourie HR, Ghorra C, Rassy M, Kesserouani C, Kattan J
Asian Pac J Cancer Prev 2016;17(5):2679-81. PMID: 27268650

Clinical prediction guides

Neuroendocrine neoplasms of the gallbladder: early detection and surgery is key to improved outcome.
Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
Prognostic factors for relapse in resected gastroenteropancreatic neuroendocrine neoplasms: A systematic review and meta-analysis.
Broadbent R, Wheatley R, Stajer S, Jacobs T, Lamarca A, Hubner RA, Valle JW, Amir E, McNamara MG
Cancer Treat Rev 2021 Dec;101:102299. Epub 2021 Oct 11 doi: 10.1016/j.ctrv.2021.102299. PMID: 34662810
AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review.
Shah SC, Piazuelo MB, Kuipers EJ, Li D
Gastroenterology 2021 Oct;161(4):1325-1332.e7. Epub 2021 Aug 26 doi: 10.1053/j.gastro.2021.06.078. PMID: 34454714Free PMC Article
A Low-Grade Appendiceal Mucinous Neoplasia and Neuroendocrine Appendiceal Collision Tumor: A Case Report and Review of the Literature.
Villa M, Sforza D, Siragusa L, Guida AM, Ciancio Manuelli M, Pirozzi BM, Pocci M, Palmieri G, Grande M
Am J Case Rep 2021 Feb 23;22:e927876. doi: 10.12659/AJCR.927876. PMID: 33619239Free PMC Article
Role of Staging in Patients with Small Intestinal Neuroendocrine Tumours.
Clift AK, Faiz O, Al-Nahhas A, Bockisch A, Liedke MO, Schloericke E, Wasan H, Martin J, Ziprin P, Moorthy K, Frilling A
J Gastrointest Surg 2016 Jan;20(1):180-8; discussion 188. Epub 2015 Sep 22 doi: 10.1007/s11605-015-2953-6. PMID: 26394880

Recent systematic reviews

Prognostic factors for relapse in resected gastroenteropancreatic neuroendocrine neoplasms: A systematic review and meta-analysis.
Broadbent R, Wheatley R, Stajer S, Jacobs T, Lamarca A, Hubner RA, Valle JW, Amir E, McNamara MG
Cancer Treat Rev 2021 Dec;101:102299. Epub 2021 Oct 11 doi: 10.1016/j.ctrv.2021.102299. PMID: 34662810
Histopathological diagnosis of appendiceal neuroendocrine neoplasms: when to perform a right hemicolectomy? A systematic review and meta-analysis.
Ricci C, Ingaldi C, Alberici L, Brighi N, Santini D, Mosconi C, Ambrosini V, Campana D, Minni F, Casadei R
Endocrine 2019 Dec;66(3):460-466. Epub 2019 Jun 21 doi: 10.1007/s12020-019-01984-z. PMID: 31227991

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