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Advanced Digestive System Neuroendocrine Neoplasm

MedGen UID:
1672664
•Concept ID:
C4764039
•
Neoplastic Process
Synonym: Advanced Gastroenteropancreatic Neuroendocrine Neoplasm

Definition

A digestive system neuroendocrine neoplasm that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Professional guidelines

PubMed

Updates in the diagnosis and management of small-bowel tumors.
Vlachou E, Koffas A, Toumpanakis C, Keuchel M
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101860. Epub 2023 Aug 12 doi: 10.1016/j.bpg.2023.101860. PMID: 37652650
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
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

Recent clinical studies

Etiology

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
(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
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
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
Clinical manifestations of chronic atrophic gastritis.
Rodriguez-Castro KI, Franceschi M, Noto A, Miraglia C, Nouvenne A, Leandro G, Meschi T, De' Angelis GL, Di Mario F
Acta Biomed 2018 Dec 17;89(8-S):88-92. doi: 10.23750/abm.v89i8-S.7921. PMID: 30561424Free PMC Article

Diagnosis

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
Small bowel radiology.
Olson MC, Navin PJ, Welle CL, Goenka AH
Curr Opin Gastroenterol 2021 May 1;37(3):267-274. doi: 10.1097/MOG.0000000000000719. PMID: 33591028
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
Clinical manifestations of chronic atrophic gastritis.
Rodriguez-Castro KI, Franceschi M, Noto A, Miraglia C, Nouvenne A, Leandro G, Meschi T, De' Angelis GL, Di Mario F
Acta Biomed 2018 Dec 17;89(8-S):88-92. doi: 10.23750/abm.v89i8-S.7921. PMID: 30561424Free 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
EUS-guided intra-tumoral therapies.
Marc B, Monino L, Rimbas M
Best Pract Res Clin Gastroenterol 2022 Sep-Dec;60-61:101817. Epub 2022 Dec 14 doi: 10.1016/j.bpg.2022.101817. PMID: 36577536
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
(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

Prospective, Multi-Institutional, Real-Time Next-Generation Sequencing of Pancreatic Cyst Fluid Reveals Diverse Genomic Alterations That Improve the Clinical Management of Pancreatic Cysts.
Paniccia A, Polanco PM, Boone BA, Wald AI, McGrath K, Brand RE, Khalid A, Kubiliun N, O'Broin-Lennon AM, Park WG, Klapman J, Tharian B, Inamdar S, Fasanella K, Nasr J, Chennat J, Das R, DeWitt J, Easler JJ, Bick B, Singh H, Fairley KJ, Sarkaria S, Sawas T, Skef W, Slivka A, Tavakkoli A, Thakkar S, Kim V, Vanderveldt HD, Richardson A, Wallace MB, Brahmbhatt B, Engels M, Gabbert C, Dugum M, El-Dika S, Bhat Y, Ramrakhiani S, Bakis G, Rolshud D, Millspaugh G, Tielleman T, Schmidt C, Mansour J, Marsh W, Ongchin M, Centeno B, Monaco SE, Ohori NP, Lajara S, Thompson ED, Hruban RH, Bell PD, Smith K, Permuth JB, Vandenbussche C, Ernst W, Grupillo M, Kaya C, Hogg M, He J, Wolfgang CL, Lee KK, Zeh H, Zureikat A, Nikiforova MN, Singhi AD
Gastroenterology 2023 Jan;164(1):117-133.e7. Epub 2022 Oct 6 doi: 10.1053/j.gastro.2022.09.028. PMID: 36209796Free 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
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
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

Clinical prediction guides

Prospective, Multi-Institutional, Real-Time Next-Generation Sequencing of Pancreatic Cyst Fluid Reveals Diverse Genomic Alterations That Improve the Clinical Management of Pancreatic Cysts.
Paniccia A, Polanco PM, Boone BA, Wald AI, McGrath K, Brand RE, Khalid A, Kubiliun N, O'Broin-Lennon AM, Park WG, Klapman J, Tharian B, Inamdar S, Fasanella K, Nasr J, Chennat J, Das R, DeWitt J, Easler JJ, Bick B, Singh H, Fairley KJ, Sarkaria S, Sawas T, Skef W, Slivka A, Tavakkoli A, Thakkar S, Kim V, Vanderveldt HD, Richardson A, Wallace MB, Brahmbhatt B, Engels M, Gabbert C, Dugum M, El-Dika S, Bhat Y, Ramrakhiani S, Bakis G, Rolshud D, Millspaugh G, Tielleman T, Schmidt C, Mansour J, Marsh W, Ongchin M, Centeno B, Monaco SE, Ohori NP, Lajara S, Thompson ED, Hruban RH, Bell PD, Smith K, Permuth JB, Vandenbussche C, Ernst W, Grupillo M, Kaya C, Hogg M, He J, Wolfgang CL, Lee KK, Zeh H, Zureikat A, Nikiforova MN, Singhi AD
Gastroenterology 2023 Jan;164(1):117-133.e7. Epub 2022 Oct 6 doi: 10.1053/j.gastro.2022.09.028. PMID: 36209796Free PMC Article
EUS-guided intra-tumoral therapies.
Marc B, Monino L, Rimbas M
Best Pract Res Clin Gastroenterol 2022 Sep-Dec;60-61:101817. Epub 2022 Dec 14 doi: 10.1016/j.bpg.2022.101817. PMID: 36577536
(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
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
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

Management and outcomes of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A systematic review and pooled analysis of 56 patients.
Ziogas IA, Rallis KS, Tasoudis PT, Moris D, Schulick RD, Del Chiaro M
Eur J Surg Oncol 2023 Apr;49(4):682-687. Epub 2023 Jan 9 doi: 10.1016/j.ejso.2023.01.005. PMID: 36646615
Systemic and liver-directed therapies in metastatic uveal melanoma: state-of-the-art and novel perspectives.
Comito F, Marchese PV, Ricci AD, Tober N, Peterle C, Sperandi F, Melotti B
Future Oncol 2021 Nov;17(33):4583-4606. Epub 2021 Aug 25 doi: 10.2217/fon-2021-0318. PMID: 34431316
Everolimus, lutetium-177 DOTATATE and sunitinib for advanced, unresectable or metastatic neuroendocrine tumours with disease progression: a systematic review and cost-effectiveness analysis.
Mujica-Mota R, Varley-Campbell J, Tikhonova I, Cooper C, Griffin E, Haasova M, Peters J, Lucherini S, Talens-Bou J, Long L, Sherriff D, Napier M, Ramage J, Hoyle M
Health Technol Assess 2018 Sep;22(49):1-326. doi: 10.3310/hta22490. PMID: 30209002Free PMC Article
Role of palliative resection of the primary tumour in advanced pancreatic and small intestinal neuroendocrine tumours: A systematic review and meta-analysis.
Almond LM, Hodson J, Ford SJ, Gourevitch D, Roberts KJ, Shah T, Isaac J, Desai A
Eur J Surg Oncol 2017 Oct;43(10):1808-1815. Epub 2017 May 25 doi: 10.1016/j.ejso.2017.05.016. PMID: 28583792
Rectal carcinoids: a systematic review.
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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