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Intestinal Neuroendocrine Tumor

MedGen UID:
474162
•Concept ID:
C3272529
•
Neoplastic Process
Synonyms: Intestinal NET; Intestinal Well Differentiated Endocrine Tumor; Intestinal Well Differentiated Endocrine Tumor/Carcinoma

Definition

A well differentiated, low, intermediate, or high grade neoplasm with neuroendocrine differentiation that arises from the small or large intestine. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntestinal Neuroendocrine Tumor

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
Gastroenteropancreatic neuroendocrine neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.
Pavel M, Öberg K, Falconi M, Krenning EP, Sundin A, Perren A, Berruti A; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2020 Jul;31(7):844-860. Epub 2020 Apr 6 doi: 10.1016/j.annonc.2020.03.304. PMID: 32272208
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

Recent clinical studies

Etiology

Genome-Wide Association Study Identifies 4 Novel Risk Loci for Small Intestinal Neuroendocrine Tumors Including a Missense Mutation in LGR5.
Giri AK, Aavikko M, Wartiovaara L, Lemmetyinen T, Karjalainen J, Mehtonen J, Palin K, Välimäki N, Tamlander M, Saikkonen R, Karhu A, Morgunova E, Sun B, Runz H, Palta P, Luo S, Joensuu H, Mäkelä TP, Kostiainen I, Schalin-Jäntti C, FinnGen, Palotie A, Aaltonen LA, Ollila S, Daly MJ
Gastroenterology 2023 Oct;165(4):861-873. Epub 2023 Jul 14 doi: 10.1053/j.gastro.2023.06.031. PMID: 37453564
Long-term organoid culture of a small intestinal neuroendocrine tumor.
D'Agosto S, Fiorini E, Pezzini F, Delfino P, Simbolo M, Vicentini C, Andreani S, Capelli P, Rusev B, Lawlor RT, Bassi C, Landoni L, Pea A, Luchini C, Scarpa A, Corbo V
Front Endocrinol (Lausanne) 2023;14:999792. Epub 2023 Apr 4 doi: 10.3389/fendo.2023.999792. PMID: 37082125Free PMC Article
Surgical Management of the Primary Tumor in Stage IV Small Intestinal Neuroendocrine Tumors: To Operate or Not to Operate, That Is the Question.
Dijke K, Kuhlmann KFD, Levy S, Tesselaar MET
Curr Oncol Rep 2023 Jun;25(6):679-688. Epub 2023 Apr 1 doi: 10.1007/s11912-023-01405-5. PMID: 37004701
Number, not size, of mesenteric tumor deposits affects prognosis of small intestinal well-differentiated neuroendocrine tumors.
Gonzalez RS, Cates JMM, Shi C
Mod Pathol 2018 Oct;31(10):1560-1566. Epub 2018 May 24 doi: 10.1038/s41379-018-0075-x. PMID: 29795438
Smoking, alcohol and family history of cancer as risk factors for small intestinal neuroendocrine tumors: a systematic review and meta-analysis.
Haugvik SP, Basim Ibrahim I, Hedenström P, Valente R, Hayes AJ, Siuka D, Gladhaug IP, Capurso G
Scand J Gastroenterol 2017 Aug;52(8):797-802. Epub 2017 Apr 10 doi: 10.1080/00365521.2017.1310290. PMID: 28394712

Diagnosis

Genome-Wide Association Study Identifies 4 Novel Risk Loci for Small Intestinal Neuroendocrine Tumors Including a Missense Mutation in LGR5.
Giri AK, Aavikko M, Wartiovaara L, Lemmetyinen T, Karjalainen J, Mehtonen J, Palin K, Välimäki N, Tamlander M, Saikkonen R, Karhu A, Morgunova E, Sun B, Runz H, Palta P, Luo S, Joensuu H, Mäkelä TP, Kostiainen I, Schalin-Jäntti C, FinnGen, Palotie A, Aaltonen LA, Ollila S, Daly MJ
Gastroenterology 2023 Oct;165(4):861-873. Epub 2023 Jul 14 doi: 10.1053/j.gastro.2023.06.031. PMID: 37453564
Surgical Management of the Primary Tumor in Stage IV Small Intestinal Neuroendocrine Tumors: To Operate or Not to Operate, That Is the Question.
Dijke K, Kuhlmann KFD, Levy S, Tesselaar MET
Curr Oncol Rep 2023 Jun;25(6):679-688. Epub 2023 Apr 1 doi: 10.1007/s11912-023-01405-5. PMID: 37004701
Exposure to nonanoic acid alters small intestinal neuroendocrine tumor phenotype.
Almobarak B, Amlani V, Inge L, Hofving T, Muth A, Nilsson O, Johansson M, Arvidsson Y, Elias E
BMC Cancer 2023 Mar 23;23(1):267. doi: 10.1186/s12885-023-10722-8. PMID: 36959559Free PMC Article
Primary small intestinal neuroendocrine tumors are highly prevalent and often multiple before metastatic disease develops.
Eriksson J, Norlén O, Ögren M, Garmo H, Ihre-Lundgren C, Hellman P
Scand J Surg 2021 Mar;110(1):44-50. Epub 2019 Oct 7 doi: 10.1177/1457496919874484. PMID: 31587594
Unexpected Finding of a Small Intestinal Neuroendocrine Tumor: A Case Report and Literature Review.
Patanè E, Sgardello SD, Guendil B, Fournier I, Abbassi Z
Am J Case Rep 2020 Jan 28;21:e917759. doi: 10.12659/AJCR.917759. PMID: 31988272Free PMC Article

Therapy

Radioguided surgery with β(-) radiation in pancreatic Neuroendocrine Tumors: a feasibility study.
Collamati F, Maccora D, Alfieri S, Bocci V, Cartoni A, Collarino A, Simoni M, Fischetti M, Fratoddi I, Giordano A, Mancini-Terracciano C, Mirabelli R, Morganti S, Quero G, Rotili D, Scotognella T, Solfaroli Camillocci E, Traini G, Venditti I, Faccini R
Sci Rep 2020 Mar 4;10(1):4015. doi: 10.1038/s41598-020-61075-2. PMID: 32132632Free PMC Article
Ex vivo activity of cytotoxic drugs and targeted agents in small intestinal neuroendocrine tumors.
Daskalakis K, Norlén O, Karakatsanis A, Hellman P, Larsson R, Nygren P, Stålberg P
Endocr Relat Cancer 2018 Apr;25(4):471-480. Epub 2018 Feb 12 doi: 10.1530/ERC-17-0404. PMID: 29440231
Smoking, alcohol and family history of cancer as risk factors for small intestinal neuroendocrine tumors: a systematic review and meta-analysis.
Haugvik SP, Basim Ibrahim I, Hedenström P, Valente R, Hayes AJ, Siuka D, Gladhaug IP, Capurso G
Scand J Gastroenterol 2017 Aug;52(8):797-802. Epub 2017 Apr 10 doi: 10.1080/00365521.2017.1310290. PMID: 28394712
Somatostatin Analogs Treated Small Intestinal Neuroendocrine Tumor Patients Circulating MicroRNAs.
Li SC, Khan M, Caplin M, Meyer T, Öberg K, Giandomenico V
PLoS One 2015;10(5):e0125553. Epub 2015 May 5 doi: 10.1371/journal.pone.0125553. PMID: 25942502Free PMC Article
Differential cytotoxicity of novel somatostatin and dopamine chimeric compounds on bronchopulmonary and small intestinal neuroendocrine tumor cell lines.
Kidd M, Drozdov I, Joseph R, Pfragner R, Culler M, Modlin I
Cancer 2008 Aug 15;113(4):690-700. doi: 10.1002/cncr.23700. PMID: 18618499Free PMC Article

Prognosis

Long-term organoid culture of a small intestinal neuroendocrine tumor.
D'Agosto S, Fiorini E, Pezzini F, Delfino P, Simbolo M, Vicentini C, Andreani S, Capelli P, Rusev B, Lawlor RT, Bassi C, Landoni L, Pea A, Luchini C, Scarpa A, Corbo V
Front Endocrinol (Lausanne) 2023;14:999792. Epub 2023 Apr 4 doi: 10.3389/fendo.2023.999792. PMID: 37082125Free PMC Article
Primary small intestinal neuroendocrine tumors are highly prevalent and often multiple before metastatic disease develops.
Eriksson J, Norlén O, Ögren M, Garmo H, Ihre-Lundgren C, Hellman P
Scand J Surg 2021 Mar;110(1):44-50. Epub 2019 Oct 7 doi: 10.1177/1457496919874484. PMID: 31587594
Unexpected Finding of a Small Intestinal Neuroendocrine Tumor: A Case Report and Literature Review.
Patanè E, Sgardello SD, Guendil B, Fournier I, Abbassi Z
Am J Case Rep 2020 Jan 28;21:e917759. doi: 10.12659/AJCR.917759. PMID: 31988272Free PMC Article
Number, not size, of mesenteric tumor deposits affects prognosis of small intestinal well-differentiated neuroendocrine tumors.
Gonzalez RS, Cates JMM, Shi C
Mod Pathol 2018 Oct;31(10):1560-1566. Epub 2018 May 24 doi: 10.1038/s41379-018-0075-x. PMID: 29795438
Somatostatin Analogs Treated Small Intestinal Neuroendocrine Tumor Patients Circulating MicroRNAs.
Li SC, Khan M, Caplin M, Meyer T, Öberg K, Giandomenico V
PLoS One 2015;10(5):e0125553. Epub 2015 May 5 doi: 10.1371/journal.pone.0125553. PMID: 25942502Free PMC Article

Clinical prediction guides

Long-term organoid culture of a small intestinal neuroendocrine tumor.
D'Agosto S, Fiorini E, Pezzini F, Delfino P, Simbolo M, Vicentini C, Andreani S, Capelli P, Rusev B, Lawlor RT, Bassi C, Landoni L, Pea A, Luchini C, Scarpa A, Corbo V
Front Endocrinol (Lausanne) 2023;14:999792. Epub 2023 Apr 4 doi: 10.3389/fendo.2023.999792. PMID: 37082125Free PMC Article
Exposure to nonanoic acid alters small intestinal neuroendocrine tumor phenotype.
Almobarak B, Amlani V, Inge L, Hofving T, Muth A, Nilsson O, Johansson M, Arvidsson Y, Elias E
BMC Cancer 2023 Mar 23;23(1):267. doi: 10.1186/s12885-023-10722-8. PMID: 36959559Free PMC Article
Ex vivo activity of cytotoxic drugs and targeted agents in small intestinal neuroendocrine tumors.
Daskalakis K, Norlén O, Karakatsanis A, Hellman P, Larsson R, Nygren P, Stålberg P
Endocr Relat Cancer 2018 Apr;25(4):471-480. Epub 2018 Feb 12 doi: 10.1530/ERC-17-0404. PMID: 29440231
Somatostatin Analogs Treated Small Intestinal Neuroendocrine Tumor Patients Circulating MicroRNAs.
Li SC, Khan M, Caplin M, Meyer T, Öberg K, Giandomenico V
PLoS One 2015;10(5):e0125553. Epub 2015 May 5 doi: 10.1371/journal.pone.0125553. PMID: 25942502Free PMC Article
A nomogram to assess small-intestinal neuroendocrine tumor ('carcinoid') survival.
Modlin IM, Gustafsson BI, Pavel M, Svejda B, Lawrence B, Kidd M
Neuroendocrinology 2010;92(3):143-57. Epub 2010 Aug 23 doi: 10.1159/000319784. PMID: 20733279

Recent systematic reviews

Smoking, alcohol and family history of cancer as risk factors for small intestinal neuroendocrine tumors: a systematic review and meta-analysis.
Haugvik SP, Basim Ibrahim I, Hedenström P, Valente R, Hayes AJ, Siuka D, Gladhaug IP, Capurso G
Scand J Gastroenterol 2017 Aug;52(8):797-802. Epub 2017 Apr 10 doi: 10.1080/00365521.2017.1310290. PMID: 28394712

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