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Digestive System Neuroendocrine Tumor G1

MedGen UID:
67386
•Concept ID:
C0220620
•
Neoplastic Process
Synonyms: Carcinoid Tumor of Digestive System; Carcinoid Tumor of Gastrointestinal System; Carcinoid tumor of gastrointestinal tract; Carcinoid Tumor of GI System; Carcinoid Tumor of the Digestive System; Carcinoid Tumor of the Gastrointestinal System; Carcinoid Tumor of the GI System; Digestive Carcinoid Tumor; Digestive System Carcinoid Tumor; Gastrointestinal Carcinoid Tumor; Gastrointestinal NET G1; Gastrointestinal Neuroendocrine Tumor G1; Gastrointestinal System Carcinoid Tumor; GI Carcinoid Tumor
SNOMED CT: Carcinoid tumor of gastrointestinal tract (428701004)

Definition

A well differentiated neuroendocrine neoplasm of low grade arising from the digestive system. It is composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with small vesicular nuclei. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent. The tumor can occur anywhere in the digestive system; approximately 90% arise in the appendix. [from NCI]

Professional guidelines

PubMed

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
The Role of Liver Transplantation in the Treatment of Liver Metastases from Neuroendocrine Tumors.
Citterio D, Coppa J, Sposito C, Busset MDD, Virdis M, Pezzoli I, Mazzaferro V
Curr Treat Options Oncol 2023 Nov;24(11):1651-1665. Epub 2023 Oct 26 doi: 10.1007/s11864-023-01124-w. PMID: 37882889Free 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

Transanal minimally invasive surgery for rectal neuroendocrine tumors.
Hayashi S, Takayama T, Ikarashi M, Hagiwara K, Matsuno Y, Suzuki T
Surg Endosc 2021 Dec;35(12):6746-6753. Epub 2020 Nov 25 doi: 10.1007/s00464-020-08178-z. PMID: 33237462
Classification and Prognostic Stratification of Bronchopulmonary Neuroendocrine Neoplasms.
Oka N, Kasajima A, Konukiewitz B, Sakurada A, Okada Y, Kameya T, Weichert W, Ishikawa Y, Suzuki H, Sasano H, Klöppel G
Neuroendocrinology 2020;110(5):393-403. Epub 2019 Aug 19 doi: 10.1159/000502776. PMID: 31422400
Pediatric gastroenteropancreatic neuroendocrine tumor: A case report and review of the literature.
Gaiani F, de'Angelis N, Minelli R, Kayali S, Carra MC, de'Angelis GL
Medicine (Baltimore) 2019 Sep;98(37):e17154. doi: 10.1097/MD.0000000000017154. PMID: 31517859Free PMC Article
Surgical treatment of extra-appendiceal colorectal neuroendocrine tumors.
Maryański J, Cyran-Chlebicka A, Szczepankiewicz B, Cebulski W, Słodkowski M, Wroński M
Pol Przegl Chir 2018 May 16;90(3):7-12. doi: 10.5604/01.3001.0011.8164. PMID: 30015320
Gastric neuroendocrine neoplasms and related precursor lesions.
La Rosa S, Vanoli A
J Clin Pathol 2014 Nov;67(11):938-48. Epub 2014 Jul 22 doi: 10.1136/jclinpath-2014-202515. PMID: 25053544

Diagnosis

G1-Neuroendocrine tumor of gallbladder with unique dendritic morphology.
Ichimaru C, Ishii T, Katanuma A
J Hepatobiliary Pancreat Sci 2022 Oct;29(10):e100-e103. Epub 2022 May 5 doi: 10.1002/jhbp.1153. PMID: 35510353
Transanal minimally invasive surgery for rectal neuroendocrine tumors.
Hayashi S, Takayama T, Ikarashi M, Hagiwara K, Matsuno Y, Suzuki T
Surg Endosc 2021 Dec;35(12):6746-6753. Epub 2020 Nov 25 doi: 10.1007/s00464-020-08178-z. PMID: 33237462
Pediatric gastroenteropancreatic neuroendocrine tumor: A case report and review of the literature.
Gaiani F, de'Angelis N, Minelli R, Kayali S, Carra MC, de'Angelis GL
Medicine (Baltimore) 2019 Sep;98(37):e17154. doi: 10.1097/MD.0000000000017154. PMID: 31517859Free PMC Article
Recent Updates on Neuroendocrine Tumors From the Gastrointestinal and Pancreatobiliary Tracts.
Kim JY, Hong SM
Arch Pathol Lab Med 2016 May;140(5):437-48. doi: 10.5858/arpa.2015-0314-RA. PMID: 27128301
Gastric neuroendocrine neoplasms and related precursor lesions.
La Rosa S, Vanoli A
J Clin Pathol 2014 Nov;67(11):938-48. Epub 2014 Jul 22 doi: 10.1136/jclinpath-2014-202515. PMID: 25053544

Therapy

Solid pseudopapillary neoplasm of pancreas: Two case reports.
Gurzu S, Bara T, Sincu M, Gabos S, Vlad DM, Bara T Jr, Beres H, Jung I
Medicine (Baltimore) 2019 Jul;98(29):e16455. doi: 10.1097/MD.0000000000016455. PMID: 31335701Free PMC Article
Clinical Usefulness of Somatostatin Receptor Scintigraphy in Japanese Patients with Gastroenteropancreatic Neuroendocrine Tumors.
Hasegawa S, Kobayashi N, Tokuhisa M, Goto A, Takano S, Takada Y, Kaneta T, Mori R, Matsuyama R, Endo I, Yamanaka S, Nakajima A, Inoue T, Ichikawa Y
Digestion 2017;96(1):13-20. Epub 2017 Jun 14 doi: 10.1159/000470838. PMID: 28609761
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
Digestive Neuroendocrine Tumor Distribution and Characteristics According to the 2010 WHO Classification: a Single Institution Experience in Lebanon.
Kourie HR, Ghorra C, Rassy M, Kesserouani C, Kattan J
Asian Pac J Cancer Prev 2016;17(5):2679-81. PMID: 27268650

Prognosis

The Role of Liver Transplantation in the Treatment of Liver Metastases from Neuroendocrine Tumors.
Citterio D, Coppa J, Sposito C, Busset MDD, Virdis M, Pezzoli I, Mazzaferro V
Curr Treat Options Oncol 2023 Nov;24(11):1651-1665. Epub 2023 Oct 26 doi: 10.1007/s11864-023-01124-w. PMID: 37882889Free 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
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
Recent Updates on Neuroendocrine Tumors From the Gastrointestinal and Pancreatobiliary Tracts.
Kim JY, Hong SM
Arch Pathol Lab Med 2016 May;140(5):437-48. doi: 10.5858/arpa.2015-0314-RA. PMID: 27128301
Gastric neuroendocrine neoplasms and related precursor lesions.
La Rosa S, Vanoli A
J Clin Pathol 2014 Nov;67(11):938-48. Epub 2014 Jul 22 doi: 10.1136/jclinpath-2014-202515. PMID: 25053544

Clinical prediction guides

The Role of Liver Transplantation in the Treatment of Liver Metastases from Neuroendocrine Tumors.
Citterio D, Coppa J, Sposito C, Busset MDD, Virdis M, Pezzoli I, Mazzaferro V
Curr Treat Options Oncol 2023 Nov;24(11):1651-1665. Epub 2023 Oct 26 doi: 10.1007/s11864-023-01124-w. PMID: 37882889Free PMC Article
Clinical outcomes and risk factors associated with poor prognosis after endoscopic resection of 10-20 mm rectal neuroendocrine tumors: a multicenter, retrospective study of 10-year experience.
Goo JJ, Baek DH, Kim HW, Lee HS, Lee JY, Park SB, Song GA, Lee SH, Lee JH; Busan Ulsan Gyeongnam Intestinal Study Group Society (BIGS)
Surg Endosc 2023 Jul;37(7):5196-5204. Epub 2023 Mar 22 doi: 10.1007/s00464-023-09999-4. PMID: 36947224
Transanal minimally invasive surgery for rectal neuroendocrine tumors.
Hayashi S, Takayama T, Ikarashi M, Hagiwara K, Matsuno Y, Suzuki T
Surg Endosc 2021 Dec;35(12):6746-6753. Epub 2020 Nov 25 doi: 10.1007/s00464-020-08178-z. PMID: 33237462
Molecular alterations in sporadic pancreatic neuroendocrine microadenomas.
Hadano A, Hirabayashi K, Yamada M, Kawanishi A, Takanashi Y, Kawaguchi Y, Nakagohri T, Nakamura N, Mine T
Pancreatology 2016 May-Jun;16(3):411-5. Epub 2016 Feb 9 doi: 10.1016/j.pan.2016.01.011. PMID: 26905832
Grading the neuroendocrine tumors of the lung: an evidence-based proposal.
Rindi G, Klersy C, Inzani F, Fellegara G, Ampollini L, Ardizzoni A, Campanini N, Carbognani P, De Pas TM, Galetta D, Granone PL, Righi L, Rusca M, Spaggiari L, Tiseo M, Viale G, Volante M, Papotti M, Pelosi G
Endocr Relat Cancer 2014 Feb;21(1):1-16. Epub 2013 Dec 16 doi: 10.1530/ERC-13-0246. PMID: 24344249

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