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Metastatic Midgut Neuroendocrine Tumor

MedGen UID:
1716948
•Concept ID:
C5238465
•
Neoplastic Process

Definition

A midgut neuroendocrine tumor that has spread from its original site of growth to another anatomic site. [from NCI]

Professional guidelines

PubMed

Somatostatin Analogues in the Treatment of Neuroendocrine Tumors: Past, Present and Future.
Stueven AK, Kayser A, Wetz C, Amthauer H, Wree A, Tacke F, Wiedenmann B, Roderburg C, Jann H
Int J Mol Sci 2019 Jun 22;20(12) doi: 10.3390/ijms20123049. PMID: 31234481Free PMC Article
Advances and Current Concepts in the Medical Management of Gastroenteropancreatic Neuroendocrine Neoplasms.
Alexandraki KI, Karapanagioti A, Karoumpalis I, Boutzios G, Kaltsas GA
Biomed Res Int 2017;2017:9856140. Epub 2017 Nov 19 doi: 10.1155/2017/9856140. PMID: 29349087Free PMC Article
Management of neuroendocrine tumors.
Chung C
Am J Health Syst Pharm 2016 Nov 1;73(21):1729-1744. doi: 10.2146/ajhp150373. PMID: 27769969

Recent clinical studies

Etiology

(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
Phase 3 Trial of (177)Lu-Dotatate for Midgut Neuroendocrine Tumors.
Strosberg J, El-Haddad G, Wolin E, Hendifar A, Yao J, Chasen B, Mittra E, Kunz PL, Kulke MH, Jacene H, Bushnell D, O'Dorisio TM, Baum RP, Kulkarni HR, Caplin M, Lebtahi R, Hobday T, Delpassand E, Van Cutsem E, Benson A, Srirajaskanthan R, Pavel M, Mora J, Berlin J, Grande E, Reed N, Seregni E, Öberg K, Lopera Sierra M, Santoro P, Thevenet T, Erion JL, Ruszniewski P, Kwekkeboom D, Krenning E; NETTER-1 Trial Investigators
N Engl J Med 2017 Jan 12;376(2):125-135. doi: 10.1056/NEJMoa1607427. PMID: 28076709Free PMC Article
Placebo-Controlled, Double-Blind, Prospective, Randomized Study on the Effect of Octreotide LAR in the Control of Tumor Growth in Patients with Metastatic Neuroendocrine Midgut Tumors (PROMID): Results of Long-Term Survival.
Rinke A, Wittenberg M, Schade-Brittinger C, Aminossadati B, Ronicke E, Gress TM, Müller HH, Arnold R; PROMID Study Group
Neuroendocrinology 2017;104(1):26-32. Epub 2016 Jan 6 doi: 10.1159/000443612. PMID: 26731483
Lanreotide in metastatic enteropancreatic neuroendocrine tumors.
Caplin ME, Pavel M, Ćwikła JB, Phan AT, Raderer M, Sedláčková E, Cadiot G, Wolin EM, Capdevila J, Wall L, Rindi G, Langley A, Martinez S, Blumberg J, Ruszniewski P; CLARINET Investigators
N Engl J Med 2014 Jul 17;371(3):224-33. doi: 10.1056/NEJMoa1316158. PMID: 25014687
Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group.
Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R; PROMID Study Group
J Clin Oncol 2009 Oct 1;27(28):4656-63. Epub 2009 Aug 24 doi: 10.1200/JCO.2009.22.8510. PMID: 19704057

Diagnosis

Value of ileus-prophylactic surgery for metastatic neuroendocrine midgut tumours.
Weber F, Dralle H
Best Pract Res Clin Endocrinol Metab 2019 Oct;33(5):101342. Epub 2019 Sep 25 doi: 10.1016/j.beem.2019.101342. PMID: 31628078
Somatostatin Analogues in the Treatment of Neuroendocrine Tumors: Past, Present and Future.
Stueven AK, Kayser A, Wetz C, Amthauer H, Wree A, Tacke F, Wiedenmann B, Roderburg C, Jann H
Int J Mol Sci 2019 Jun 22;20(12) doi: 10.3390/ijms20123049. PMID: 31234481Free PMC Article
Somatostatin Receptor 2-Targeting Compounds.
Smit Duijzentkunst DA, Kwekkeboom DJ, Bodei L
J Nucl Med 2017 Sep;58(Suppl 2):54S-60S. doi: 10.2967/jnumed.117.191015. PMID: 28864613
Gastroenteropancreatic neuroendocrine (carcinoid) tumors in children.
Johnson PR
Semin Pediatr Surg 2014 Apr;23(2):91-5. Epub 2014 Mar 14 doi: 10.1053/j.sempedsurg.2014.03.007. PMID: 24931354
Carcinoid tumors.
Robertson RG, Geiger WJ, Davis NB
Am Fam Physician 2006 Aug 1;74(3):429-34. PMID: 16913162

Therapy

(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
Somatostatin Analogues in the Treatment of Neuroendocrine Tumors: Past, Present and Future.
Stueven AK, Kayser A, Wetz C, Amthauer H, Wree A, Tacke F, Wiedenmann B, Roderburg C, Jann H
Int J Mol Sci 2019 Jun 22;20(12) doi: 10.3390/ijms20123049. PMID: 31234481Free PMC Article
Phase 3 Trial of (177)Lu-Dotatate for Midgut Neuroendocrine Tumors.
Strosberg J, El-Haddad G, Wolin E, Hendifar A, Yao J, Chasen B, Mittra E, Kunz PL, Kulke MH, Jacene H, Bushnell D, O'Dorisio TM, Baum RP, Kulkarni HR, Caplin M, Lebtahi R, Hobday T, Delpassand E, Van Cutsem E, Benson A, Srirajaskanthan R, Pavel M, Mora J, Berlin J, Grande E, Reed N, Seregni E, Öberg K, Lopera Sierra M, Santoro P, Thevenet T, Erion JL, Ruszniewski P, Kwekkeboom D, Krenning E; NETTER-1 Trial Investigators
N Engl J Med 2017 Jan 12;376(2):125-135. doi: 10.1056/NEJMoa1607427. PMID: 28076709Free PMC Article
Placebo-Controlled, Double-Blind, Prospective, Randomized Study on the Effect of Octreotide LAR in the Control of Tumor Growth in Patients with Metastatic Neuroendocrine Midgut Tumors (PROMID): Results of Long-Term Survival.
Rinke A, Wittenberg M, Schade-Brittinger C, Aminossadati B, Ronicke E, Gress TM, Müller HH, Arnold R; PROMID Study Group
Neuroendocrinology 2017;104(1):26-32. Epub 2016 Jan 6 doi: 10.1159/000443612. PMID: 26731483
Lanreotide in metastatic enteropancreatic neuroendocrine tumors.
Caplin ME, Pavel M, Ćwikła JB, Phan AT, Raderer M, Sedláčková E, Cadiot G, Wolin EM, Capdevila J, Wall L, Rindi G, Langley A, Martinez S, Blumberg J, Ruszniewski P; CLARINET Investigators
N Engl J Med 2014 Jul 17;371(3):224-33. doi: 10.1056/NEJMoa1316158. PMID: 25014687

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
Somatostatin Analogues in the Treatment of Neuroendocrine Tumors: Past, Present and Future.
Stueven AK, Kayser A, Wetz C, Amthauer H, Wree A, Tacke F, Wiedenmann B, Roderburg C, Jann H
Int J Mol Sci 2019 Jun 22;20(12) doi: 10.3390/ijms20123049. PMID: 31234481Free PMC Article
Placebo-Controlled, Double-Blind, Prospective, Randomized Study on the Effect of Octreotide LAR in the Control of Tumor Growth in Patients with Metastatic Neuroendocrine Midgut Tumors (PROMID): Results of Long-Term Survival.
Rinke A, Wittenberg M, Schade-Brittinger C, Aminossadati B, Ronicke E, Gress TM, Müller HH, Arnold R; PROMID Study Group
Neuroendocrinology 2017;104(1):26-32. Epub 2016 Jan 6 doi: 10.1159/000443612. PMID: 26731483
Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group.
Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R; PROMID Study Group
J Clin Oncol 2009 Oct 1;27(28):4656-63. Epub 2009 Aug 24 doi: 10.1200/JCO.2009.22.8510. PMID: 19704057
Carcinoid tumors.
Robertson RG, Geiger WJ, Davis NB
Am Fam Physician 2006 Aug 1;74(3):429-34. PMID: 16913162

Clinical prediction guides

Bone metastases in midgut neuroendocrine tumors: imaging characteristics, distribution, and risk factors.
Lelièvre M, Triumbari EKA, Brixi H, Perrier M, Cadiot G, Deguelte S, Morland D
Endocrine 2022 Nov;78(2):380-386. Epub 2022 Oct 7 doi: 10.1007/s12020-022-03160-2. PMID: 36203032
(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
Phase 3 Trial of (177)Lu-Dotatate for Midgut Neuroendocrine Tumors.
Strosberg J, El-Haddad G, Wolin E, Hendifar A, Yao J, Chasen B, Mittra E, Kunz PL, Kulke MH, Jacene H, Bushnell D, O'Dorisio TM, Baum RP, Kulkarni HR, Caplin M, Lebtahi R, Hobday T, Delpassand E, Van Cutsem E, Benson A, Srirajaskanthan R, Pavel M, Mora J, Berlin J, Grande E, Reed N, Seregni E, Öberg K, Lopera Sierra M, Santoro P, Thevenet T, Erion JL, Ruszniewski P, Kwekkeboom D, Krenning E; NETTER-1 Trial Investigators
N Engl J Med 2017 Jan 12;376(2):125-135. doi: 10.1056/NEJMoa1607427. PMID: 28076709Free PMC Article
Placebo-Controlled, Double-Blind, Prospective, Randomized Study on the Effect of Octreotide LAR in the Control of Tumor Growth in Patients with Metastatic Neuroendocrine Midgut Tumors (PROMID): Results of Long-Term Survival.
Rinke A, Wittenberg M, Schade-Brittinger C, Aminossadati B, Ronicke E, Gress TM, Müller HH, Arnold R; PROMID Study Group
Neuroendocrinology 2017;104(1):26-32. Epub 2016 Jan 6 doi: 10.1159/000443612. PMID: 26731483
Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group.
Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R; PROMID Study Group
J Clin Oncol 2009 Oct 1;27(28):4656-63. Epub 2009 Aug 24 doi: 10.1200/JCO.2009.22.8510. PMID: 19704057

Recent systematic reviews

Is Resection of Primary Midgut Neuroendocrine Tumors in Patients with Unresectable Metastatic Liver Disease Justified? A Systematic Review and Meta-Analysis.
Tsilimigras DI, Ntanasis-Stathopoulos I, Kostakis ID, Moris D, Schizas D, Cloyd JM, Pawlik TM
J Gastrointest Surg 2019 May;23(5):1044-1054. Epub 2019 Jan 22 doi: 10.1007/s11605-018-04094-9. PMID: 30671800
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
Systematic review of resecting primary tumor in MNETs patients with unresectable liver metastases.
Guo J, Zhang Q, Bi X, Zhou J, Li Z, Huang Z, Zhang Y, Li M, Chen X, Hu X, Yihebali C, Liang J, Liu J, Zhao J, Cai J, Zhao H
Oncotarget 2017 Mar 7;8(10):17396-17405. doi: 10.18632/oncotarget.14156. PMID: 28030811Free PMC Article
Systematic review of resection of primary midgut carcinoid tumour in patients with unresectable liver metastases.
Capurso G, Rinzivillo M, Bettini R, Boninsegna L, Delle Fave G, Falconi M
Br J Surg 2012 Nov;99(11):1480-6. Epub 2012 Sep 13 doi: 10.1002/bjs.8842. PMID: 22972490

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