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Small intestinal neuroendocrine tumor G1

MedGen UID:
91150
•Concept ID:
C0349536
•
Neoplastic Process
Synonyms: Carcinoid Tumor of Small Bowel; carcinoid tumor of small bowel; Carcinoid tumor of small intestine; Carcinoid Tumor of Small Intestine; carcinoid tumor of small intestine; Carcinoid Tumor of the Small Bowel; carcinoid tumor of the small bowel; Carcinoid Tumor of the Small Intestine; carcinoid tumor of the small intestine; carcinoid tumour of small bowel; carcinoid tumour of small intestine; carcinoid tumour of the small bowel; carcinoid tumour of the small intestine; grade 1 neuroendocrine neoplasm of small intestine; Small Bowel Carcinoid Tumor; small bowel carcinoid tumor; small bowel carcinoid tumour; Small Intestinal Carcinoid Tumor; small intestinal carcinoid tumor; small intestinal carcinoid tumour; Small Intestinal NET G1; small intestinal NET G1; Small Intestinal Neuroendocrine Tumor G1; small intestinal neuroendocrine tumor G1; small intestine carcinoid neuroendocrine tumor; small intestine carcinoid neuroendocrine tumour; Small Intestine Carcinoid Tumor; small intestine carcinoid tumor; small intestine carcinoid tumor (disease); small intestine carcinoid tumour; small intestine carcinoid tumour (disease); small intestine NET G1; small intestine neuroendocrine neoplasm G1; small intestine neuroendocrine tumor, well differentiated, low grade
SNOMED CT: Carcinoid tumor of small intestine (276818002)
 
Monarch Initiative: MONDO:0000540

Definition

A well differentiated, low grade neuroendocrine neoplasm (carcinoid tumor) that arises from the small intestine. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall intestinal neuroendocrine tumor G1

Professional guidelines

PubMed

Characteristics and treatment results of patients with gastroenteropancreatic neuroendocrine tumors in a tertiary care centre.
Bazarbashi S, Aseafan M, Elgazzar T, Alkhayat M, Alghabban A, Abdelgawad MI, Alshamsan B, Alshibany A, Elhassan T, Aljubran A, Alzahrani A, Alhindi H, Raef H
BMC Endocr Disord 2023 Apr 7;23(1):74. doi: 10.1186/s12902-023-01326-1. PMID: 37029347Free PMC Article
Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort.
Papantoniou D, Grönberg M, Thiis-Evensen E, Sorbye H, Landerholm K, Welin S, Tiensuu Janson E
Endocr Relat Cancer 2023 Mar 1;30(3) Epub 2023 Feb 14 doi: 10.1530/ERC-22-0316. PMID: 36629395Free PMC Article
Heterogeneity of grade 3 gastroenteropancreatic neuroendocrine carcinomas: New insights and treatment implications.
Fazio N, Milione M
Cancer Treat Rev 2016 Nov;50:61-67. Epub 2016 Aug 28 doi: 10.1016/j.ctrv.2016.08.006. PMID: 27636009

Recent clinical studies

Etiology

Site-specific trends in gastroenteropancreatic neuroendocrine neoplasms in Bavaria, Germany.
Grundmann N, Voigtländer S, Hakimhashemi A, Pape UF, Meyer M, Müller-Nordhorn J
Cancer Med 2023 Oct;12(19):19949-19958. Epub 2023 Sep 22 doi: 10.1002/cam4.6510. PMID: 37737059Free PMC Article
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
Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort.
Papantoniou D, Grönberg M, Thiis-Evensen E, Sorbye H, Landerholm K, Welin S, Tiensuu Janson E
Endocr Relat Cancer 2023 Mar 1;30(3) Epub 2023 Feb 14 doi: 10.1530/ERC-22-0316. PMID: 36629395Free PMC Article
Prognosis of incompletely resected small rectal neuroendocrine tumor using endoscope without additional treatment.
Cha B, Shin J, Ko WJ, Kwon KS, Kim H
BMC Gastroenterol 2022 Jun 9;22(1):293. doi: 10.1186/s12876-022-02365-z. PMID: 35681149Free PMC Article
Vitamin D deficiency and tumor aggressiveness in gastroenteropancreatic neuroendocrine tumors.
Altieri B, Barrea L, Modica R, Bottiglieri F, de Cicco F, Muscogiuri G, Circelli L, Savarese G, Di Somma C, Savastano S, Colao A, Faggiano A
Endocrine 2022 Feb;75(2):623-634. Epub 2021 Sep 17 doi: 10.1007/s12020-021-02869-w. PMID: 34533768

Diagnosis

European Neuroendocrine Tumor Society (ENETS) 2024 guidance paper for the management of well-differentiated small intestine neuroendocrine tumours.
Lamarca A, Bartsch DK, Caplin M, Kos-Kudla B, Kjaer A, Partelli S, Rinke A, Janson ET, Thirlwell C, van Velthuysen MF, Vullierme MP, Pavel M
J Neuroendocrinol 2024 Sep;36(9):e13423. Epub 2024 Jul 8 doi: 10.1111/jne.13423. PMID: 38977327
Site-specific trends in gastroenteropancreatic neuroendocrine neoplasms in Bavaria, Germany.
Grundmann N, Voigtländer S, Hakimhashemi A, Pape UF, Meyer M, Müller-Nordhorn J
Cancer Med 2023 Oct;12(19):19949-19958. Epub 2023 Sep 22 doi: 10.1002/cam4.6510. PMID: 37737059Free PMC Article
Prognosis of incompletely resected small rectal neuroendocrine tumor using endoscope without additional treatment.
Cha B, Shin J, Ko WJ, Kwon KS, Kim H
BMC Gastroenterol 2022 Jun 9;22(1):293. doi: 10.1186/s12876-022-02365-z. PMID: 35681149Free PMC Article
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
Current knowledge on diagnosis and staging of neuroendocrine tumors.
Oberg K, Castellano D
Cancer Metastasis Rev 2011 Mar;30 Suppl 1:3-7. doi: 10.1007/s10555-011-9292-1. PMID: 21311954

Therapy

Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort.
Papantoniou D, Grönberg M, Thiis-Evensen E, Sorbye H, Landerholm K, Welin S, Tiensuu Janson E
Endocr Relat Cancer 2023 Mar 1;30(3) Epub 2023 Feb 14 doi: 10.1530/ERC-22-0316. PMID: 36629395Free PMC Article
Endoscopic Resection of Ampullary Neuroendocrine Tumor.
Fukasawa H, Tounou S, Nabetani M, Michida T
Intern Med 2017;56(5):499-503. Epub 2017 Mar 1 doi: 10.2169/internalmedicine.56.7520. PMID: 28250294Free PMC Article
Heterogeneity of grade 3 gastroenteropancreatic neuroendocrine carcinomas: New insights and treatment implications.
Fazio N, Milione M
Cancer Treat Rev 2016 Nov;50:61-67. Epub 2016 Aug 28 doi: 10.1016/j.ctrv.2016.08.006. PMID: 27636009
Identification of the BRAF V600E mutation in gastroenteropancreatic neuroendocrine tumors.
Park C, Ha SY, Kim ST, Kim HC, Heo JS, Park YS, Lauwers G, Lee J, Kim KM
Oncotarget 2016 Jan 26;7(4):4024-35. doi: 10.18632/oncotarget.6602. PMID: 26684240Free PMC Article
Biotherapies for GEP-NETs.
Öberg K
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):833-41. doi: 10.1016/j.bpg.2013.01.001. PMID: 23582922

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
Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort.
Papantoniou D, Grönberg M, Thiis-Evensen E, Sorbye H, Landerholm K, Welin S, Tiensuu Janson E
Endocr Relat Cancer 2023 Mar 1;30(3) Epub 2023 Feb 14 doi: 10.1530/ERC-22-0316. PMID: 36629395Free PMC Article
Prognosis of incompletely resected small rectal neuroendocrine tumor using endoscope without additional treatment.
Cha B, Shin J, Ko WJ, Kwon KS, Kim H
BMC Gastroenterol 2022 Jun 9;22(1):293. doi: 10.1186/s12876-022-02365-z. PMID: 35681149Free PMC Article
Vitamin D deficiency and tumor aggressiveness in gastroenteropancreatic neuroendocrine tumors.
Altieri B, Barrea L, Modica R, Bottiglieri F, de Cicco F, Muscogiuri G, Circelli L, Savarese G, Di Somma C, Savastano S, Colao A, Faggiano A
Endocrine 2022 Feb;75(2):623-634. Epub 2021 Sep 17 doi: 10.1007/s12020-021-02869-w. PMID: 34533768
Genetics and Epigenetics of Gastroenteropancreatic Neuroendocrine Neoplasms.
Mafficini A, Scarpa A
Endocr Rev 2019 Apr 1;40(2):506-536. doi: 10.1210/er.2018-00160. PMID: 30657883Free 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
Clinical outcomes of gastroduodenal neuroendocrine tumors according to their WHO grade: A single-institutional retrospective analysis.
Ryu DG, Kim SJ, Choi CW, Kang DH, Kim HW, Park SB, Nam HS, Lee SH, Hwang SH
Medicine (Baltimore) 2022 Sep 16;101(37):e30397. doi: 10.1097/MD.0000000000030397. PMID: 36123854Free PMC Article
Plasma protein biomarkers for the detection of pancreatic neuroendocrine tumors and differentiation from small intestinal neuroendocrine tumors.
Thiis-Evensen E, Kjellman M, Knigge U, Gronbaek H, Schalin-Jäntti C, Welin S, Sorbye H, Del Pilar Schneider M, Belusa R; Nordic NET Biomarker Group
J Neuroendocrinol 2022 Jul;34(7):e13176. Epub 2022 Jul 13 doi: 10.1111/jne.13176. PMID: 35829662Free PMC Article
Double-Balloon Enteroscopy in Detecting Small-Bowel Neuroendocrine Neoplasms: A Single-Center Prospective Study.
Rossi RE, Elli L, Branchi F, Conte D, Massironi S
Digestion 2021;102(5):722-730. Epub 2020 Dec 23 doi: 10.1159/000511850. PMID: 33361694
Immune checkpoint markers in gastroenteropancreatic neuroendocrine neoplasia.
Bösch F, Brüwer K, Altendorf-Hofmann A, Auernhammer CJ, Spitzweg C, Westphalen CB, Boeck S, Schubert-Fritschle G, Werner J, Heinemann V, Kirchner T, Angele M, Knösel T
Endocr Relat Cancer 2019 Mar 1;26(3):293-301. doi: 10.1530/ERC-18-0494. PMID: 30608901

Recent systematic reviews

Neuroendocrine neoplasms of gastrointestinal tract and secondary primary synchronous tumors: A systematic review of case reports. Casualty or causality?
Parra-Medina R, Moreno-Lucero P, Jimenez-Moreno J, Parra-Morales AM, Romero-Rojas A
PLoS One 2019;14(5):e0216647. Epub 2019 May 14 doi: 10.1371/journal.pone.0216647. PMID: 31086397Free PMC Article

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