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Digestive system neuroendocrine neoplasm

MedGen UID:
232237
•Concept ID:
C1333799
•
Neoplastic Process
Synonyms: alimentary part of gastrointestinal system NET; alimentary part of gastrointestinal system neuroendocrine neoplasm; alimentary part of gastrointestinal system neuroendocrine tumor; alimentary part of gastrointestinal system neuroendocrine tumor, well differentiated, low or intermediate grade; alimentary part of gastrointestinal system neuroendocrine tumour; carcinoid tumor; carcinoid tumor of digestive system; carcinoid tumour; carcinoid tumour of digestive system; digestive system NET; Digestive System Neuroendocrine Neoplasm; digestive system neuroendocrine neoplasm; digestive system neuroendocrine tumor; digestive system neuroendocrine tumor, well differentiated, low or intermediate grade; digestive system neuroendocrine tumour; gastro-enteropancreatic neuroendocrine tumor; gastro-enteropancreatic neuroendocrine tumour; gastroenteropancreatic endocrine tumor; gastroenteropancreatic endocrine tumour; Gastroenteropancreatic Neuroendocrine Neoplasm; gastroenteropancreatic neuroendocrine neoplasm; Gastroenteropancreatic neuroendocrine neoplasm; Gastrointestinal Neuroendocrine Neoplasm; gastrointestinal neuroendocrine neoplasm; Gastrointestinal System Neuroendocrine Neoplasm; gastrointestinal system neuroendocrine neoplasm; GEP tumors; GEP tumours; GEP-NEN; GEP-NET; neuroendocrine neoplasm of alimentary part of gastrointestinal system; neuroendocrine neoplasm of digestive system
 
Monarch Initiative: MONDO:0024503
Orphanet: ORPHA100092

Definition

A neoplasm with neuroendocrine differentiation arising from the digestive system. It includes neuroendocrine tumors (well-differentiated neuroendocrine neoplasms) and neuroendocrine carcinomas (poorly differentiated neuroendocrine neoplasms). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDigestive system neuroendocrine neoplasm

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
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190
Evaluation and Management of Neuroendocrine Tumors of the Pancreas.
Scott AT, Howe JR
Surg Clin North Am 2019 Aug;99(4):793-814. Epub 2019 May 27 doi: 10.1016/j.suc.2019.04.014. PMID: 31255207Free PMC Article

Recent clinical studies

Etiology

Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls.
Konukiewitz B, Jesinghaus M, Kasajima A, Klöppel G
Virchows Arch 2022 Feb;480(2):247-257. Epub 2021 Oct 13 doi: 10.1007/s00428-021-03211-5. PMID: 34647171Free PMC Article
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free 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 tumors: A review of serum biomarkers, staging, and management.
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH
World J Gastroenterol 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795Free PMC Article
Evaluation and Management of Neuroendocrine Tumors of the Pancreas.
Scott AT, Howe JR
Surg Clin North Am 2019 Aug;99(4):793-814. Epub 2019 May 27 doi: 10.1016/j.suc.2019.04.014. PMID: 31255207Free PMC Article

Diagnosis

Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
What is New in the 2019 World Health Organization (WHO) Classification of Tumors of the Digestive System: Review of Selected Updates on Neuroendocrine Neoplasms, Appendiceal Tumors, and Molecular Testing.
Assarzadegan N, Montgomery E
Arch Pathol Lab Med 2021 Jun 1;145(6):664-677. doi: 10.5858/arpa.2019-0665-RA. PMID: 32233993Free PMC Article
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190
Pancreatic neuroendocrine tumors: A review of serum biomarkers, staging, and management.
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH
World J Gastroenterol 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795Free PMC Article
Evaluation and Management of Neuroendocrine Tumors of the Pancreas.
Scott AT, Howe JR
Surg Clin North Am 2019 Aug;99(4):793-814. Epub 2019 May 27 doi: 10.1016/j.suc.2019.04.014. PMID: 31255207Free PMC Article

Therapy

Progress in immunotherapy for neuroendocrine neoplasm of the digestive system.
Pan WX, Zhang XM, Hao SL, Han W
World J Gastroenterol 2023 Jul 14;29(26):4174-4185. doi: 10.3748/wjg.v29.i26.4174. PMID: 37475845Free PMC Article
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
Claudin-18.
Wong MT, Singhi AD, Larson BK, Huynh CAT, Balzer BL, Burch M, Dhall D, Gangi A, Gong J, Guindi M, Hendifar AE, Kim SA, de Peralta-Venturina M, Waters KM
Arch Pathol Lab Med 2022 May 1;147(5):559-567. doi: 10.5858/arpa.2021-0428-OA. PMID: 35976638
(177)Lu-Dotatate plus long-acting octreotide versus high‑dose long-acting octreotide in patients with midgut neuroendocrine tumours (NETTER-1): final overall survival and long-term safety results from an open-label, randomised, controlled, phase 3 trial.
Strosberg JR, Caplin ME, Kunz PL, Ruszniewski PB, Bodei L, Hendifar A, Mittra E, Wolin EM, Yao JC, Pavel ME, Grande E, Van Cutsem E, Seregni E, Duarte H, Gericke G, Bartalotta A, Mariani MF, Demange A, Mutevelic S, Krenning EP; NETTER-1 investigators
Lancet Oncol 2021 Dec;22(12):1752-1763. Epub 2021 Nov 15 doi: 10.1016/S1470-2045(21)00572-6. PMID: 34793718
Pancreatic neuroendocrine tumors: A review of serum biomarkers, staging, and management.
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH
World J Gastroenterol 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795Free PMC Article

Prognosis

(177)Lu-Dotatate plus long-acting octreotide versus high‑dose long-acting octreotide in patients with midgut neuroendocrine tumours (NETTER-1): final overall survival and long-term safety results from an open-label, randomised, controlled, phase 3 trial.
Strosberg JR, Caplin ME, Kunz PL, Ruszniewski PB, Bodei L, Hendifar A, Mittra E, Wolin EM, Yao JC, Pavel ME, Grande E, Van Cutsem E, Seregni E, Duarte H, Gericke G, Bartalotta A, Mariani MF, Demange A, Mutevelic S, Krenning EP; NETTER-1 investigators
Lancet Oncol 2021 Dec;22(12):1752-1763. Epub 2021 Nov 15 doi: 10.1016/S1470-2045(21)00572-6. PMID: 34793718
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
What is New in the 2019 World Health Organization (WHO) Classification of Tumors of the Digestive System: Review of Selected Updates on Neuroendocrine Neoplasms, Appendiceal Tumors, and Molecular Testing.
Assarzadegan N, Montgomery E
Arch Pathol Lab Med 2021 Jun 1;145(6):664-677. doi: 10.5858/arpa.2019-0665-RA. PMID: 32233993Free PMC Article
Pancreatic neuroendocrine tumors: A review of serum biomarkers, staging, and management.
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH
World J Gastroenterol 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795Free PMC Article
Evaluation and Management of Neuroendocrine Tumors of the Pancreas.
Scott AT, Howe JR
Surg Clin North Am 2019 Aug;99(4):793-814. Epub 2019 May 27 doi: 10.1016/j.suc.2019.04.014. PMID: 31255207Free PMC Article

Clinical prediction guides

Narrative Review of Immunotherapy in Gastroentero-Pancreatic Neuroendocrine Neoplasms.
Kaur J, Vijayvergia N
Curr Oncol 2023 Sep 21;30(9):8653-8664. doi: 10.3390/curroncol30090627. PMID: 37754542Free PMC Article
(177)Lu-Dotatate plus long-acting octreotide versus high‑dose long-acting octreotide in patients with midgut neuroendocrine tumours (NETTER-1): final overall survival and long-term safety results from an open-label, randomised, controlled, phase 3 trial.
Strosberg JR, Caplin ME, Kunz PL, Ruszniewski PB, Bodei L, Hendifar A, Mittra E, Wolin EM, Yao JC, Pavel ME, Grande E, Van Cutsem E, Seregni E, Duarte H, Gericke G, Bartalotta A, Mariani MF, Demange A, Mutevelic S, Krenning EP; NETTER-1 investigators
Lancet Oncol 2021 Dec;22(12):1752-1763. Epub 2021 Nov 15 doi: 10.1016/S1470-2045(21)00572-6. PMID: 34793718
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 of digestive system tumours.
Washington MK, Goldberg RM, Chang GJ, Limburg P, Lam AK, Salto-Tellez M, Arends MJ, Nagtegaal ID, Klimstra DS, Rugge M, Schirmacher P, Lazar AJ, Odze RD, Carneiro F, Fukayama M, Cree IA; WHO Classification of Tumours Editorial Board
Int J Cancer 2021 Mar 1;148(5):1040-1050. Epub 2020 Nov 19 doi: 10.1002/ijc.33210. PMID: 32674220
What is New in the 2019 World Health Organization (WHO) Classification of Tumors of the Digestive System: Review of Selected Updates on Neuroendocrine Neoplasms, Appendiceal Tumors, and Molecular Testing.
Assarzadegan N, Montgomery E
Arch Pathol Lab Med 2021 Jun 1;145(6):664-677. doi: 10.5858/arpa.2019-0665-RA. PMID: 32233993Free PMC Article

Recent systematic reviews

Duodenum-Preserving Pancreatic Head Resection for Benign and Premalignant Tumors-a Systematic Review and Meta-analysis of Surgery-Associated Morbidity.
Beger HG, Mayer B, Poch B
J Gastrointest Surg 2023 Nov;27(11):2611-2627. Epub 2023 Sep 5 doi: 10.1007/s11605-023-05789-4. PMID: 37670106Free PMC Article
Completion Pancreatectomy. Indications and Outcomes: A Systematic Review.
Vouros D, Bramis K, Alexakis N, Kotsarinis V, Antonakis P, Memos N, Konstadoulakis M, Toutouzas K
Am Surg 2023 Dec;89(12):6134-6146. Epub 2023 Jun 9 doi: 10.1177/00031348231183121. PMID: 37295804
Metastasis to the rectum: A systematic review of the literature.
Janjic O, Labgaa I, Hübner M, Demartines N, Joliat GR
Eur J Surg Oncol 2022 Apr;48(4):822-833. Epub 2021 Oct 10 doi: 10.1016/j.ejso.2021.10.004. PMID: 34656391
Pancreatic Neuroendocrine Tumors and Intraductal Papillary Mucinous Neoplasm of the Pancreas: A Systematic Review.
Manuel-Vazquez A, Ramia JM, Latorre-Fragua R, Valle-Rubio A, Arteaga-Peralta V, Ramiro-Pérez C, de la Plaza-Llamas R
Pancreas 2018 May/Jun;47(5):551-555. doi: 10.1097/MPA.0000000000001048. PMID: 29683974
Risk factors for neuroendocrine neoplasms: a systematic review and meta-analysis.
Leoncini E, Carioli G, La Vecchia C, Boccia S, Rindi G
Ann Oncol 2016 Jan;27(1):68-81. Epub 2015 Oct 20 doi: 10.1093/annonc/mdv505. PMID: 26487581

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