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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

Deprez PH, Moons LMG, OʼToole D, Gincul R, Seicean A, Pimentel-Nunes P, Fernández-Esparrach G, Polkowski M, Vieth M, Borbath I, Moreels TG, Nieveen van Dijkum E, Blay JY, van Hooft JE
Endoscopy 2022 Apr;54(4):412-429. Epub 2022 Feb 18 doi: 10.1055/a-1751-5742. PMID: 35180797
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
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

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
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
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
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
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

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
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190
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
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
de Mestier L, Cros J, Neuzillet C, Hentic O, Egal A, Muller N, Bouché O, Cadiot G, Ruszniewski P, Couvelard A, Hammel P
Neuroendocrinology 2017;105(4):412-425. Epub 2017 Aug 12 doi: 10.1159/000475527. PMID: 28803232

Therapy

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
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
Vijayvergia N, Dasari A
Curr Treat Options Oncol 2020 Oct 7;21(12):96. doi: 10.1007/s11864-020-00794-0. PMID: 33029680Free PMC Article
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
Basuroy R, Srirajaskanthan R, Ramage JK
Gastroenterol Clin North Am 2016 Sep;45(3):487-507. doi: 10.1016/j.gtc.2016.04.007. PMID: 27546845

Prognosis

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
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
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
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
Basuroy R, Srirajaskanthan R, Ramage JK
Gastroenterol Clin North Am 2016 Sep;45(3):487-507. doi: 10.1016/j.gtc.2016.04.007. PMID: 27546845

Clinical prediction guides

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
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
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
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
Uccella S, La Rosa S
Histopathology 2020 Nov;77(5):700-717. Epub 2020 Sep 12 doi: 10.1111/his.14178. PMID: 32538468

Recent systematic reviews

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
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
Beger HG, Nakao A, Mayer B, Poch B
Pancreatology 2015 Mar-Apr;15(2):167-78. Epub 2015 Feb 7 doi: 10.1016/j.pan.2015.01.009. PMID: 25732271
Michalopoulos N, Papavramidis TS, Karayannopoulou G, Pliakos I, Papavramidis ST, Kanellos I
Pathol Oncol Res 2014 Oct;20(4):765-75. Epub 2014 Jun 11 doi: 10.1007/s12253-014-9808-4. PMID: 24917351
McDermott FD, Heeney A, Courtney D, Mohan H, Winter D
Surg Endosc 2014 Jul;28(7):2020-6. Epub 2014 Mar 1 doi: 10.1007/s00464-014-3430-0. PMID: 24584484

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