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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

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
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
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

Parisi A, Delaunay B, Pinterpe G, Hollebecque A, Blanc JF, Bouattour M, Assenat E, Ben Abdelghani M, Sarabi M, Niger M, Vivaldi C, Mandalà M, Palloni A, Bensi M, Garattini SK, Tougeron D, Combe P, Salati M, Rimini M, Cella CA, Tucci M, Diana A, Mori E, Longarini R, Artru P, Roth G, Evesque L, Vienne A, Turpin A, Hiret S, Bourgeois V, Herve C, Paulon R, Stacoffe M, Malka D, Neuzillet C, Edeline J, Lievre A, Guimbaud R, Chapda MCP, Rimassa L, Giampieri R, Valle J, Berardi R, Fares N
Eur J Cancer 2024 Mar;200:113587. Epub 2024 Feb 6 doi: 10.1016/j.ejca.2024.113587. PMID: 38340384
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
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
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
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

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
Parisi A, Delaunay B, Pinterpe G, Hollebecque A, Blanc JF, Bouattour M, Assenat E, Ben Abdelghani M, Sarabi M, Niger M, Vivaldi C, Mandalà M, Palloni A, Bensi M, Garattini SK, Tougeron D, Combe P, Salati M, Rimini M, Cella CA, Tucci M, Diana A, Mori E, Longarini R, Artru P, Roth G, Evesque L, Vienne A, Turpin A, Hiret S, Bourgeois V, Herve C, Paulon R, Stacoffe M, Malka D, Neuzillet C, Edeline J, Lievre A, Guimbaud R, Chapda MCP, Rimassa L, Giampieri R, Valle J, Berardi R, Fares N
Eur J Cancer 2024 Mar;200:113587. Epub 2024 Feb 6 doi: 10.1016/j.ejca.2024.113587. PMID: 38340384
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
Kim JY, Hong SM
Arch Pathol Lab Med 2016 May;140(5):437-48. doi: 10.5858/arpa.2015-0314-RA. PMID: 27128301
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

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
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
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
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
Kourie HR, Ghorra C, Rassy M, Kesserouani C, Kattan J
Asian Pac J Cancer Prev 2016;17(5):2679-81. PMID: 27268650

Prognosis

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
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
Mafficini A, Scarpa A
Endocr Rev 2019 Apr 1;40(2):506-536. doi: 10.1210/er.2018-00160. PMID: 30657883Free PMC Article
Kim JY, Hong SM
Arch Pathol Lab Med 2016 May;140(5):437-48. doi: 10.5858/arpa.2015-0314-RA. PMID: 27128301
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

Parisi A, Delaunay B, Pinterpe G, Hollebecque A, Blanc JF, Bouattour M, Assenat E, Ben Abdelghani M, Sarabi M, Niger M, Vivaldi C, Mandalà M, Palloni A, Bensi M, Garattini SK, Tougeron D, Combe P, Salati M, Rimini M, Cella CA, Tucci M, Diana A, Mori E, Longarini R, Artru P, Roth G, Evesque L, Vienne A, Turpin A, Hiret S, Bourgeois V, Herve C, Paulon R, Stacoffe M, Malka D, Neuzillet C, Edeline J, Lievre A, Guimbaud R, Chapda MCP, Rimassa L, Giampieri R, Valle J, Berardi R, Fares N
Eur J Cancer 2024 Mar;200:113587. Epub 2024 Feb 6 doi: 10.1016/j.ejca.2024.113587. PMID: 38340384
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
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
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
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

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