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Digestive System Neuroendocrine Tumor G1

MedGen UID:
67386
Concept ID:
C0220620
Neoplastic Process
Synonyms: Gastrointestinal NET G1; Gastrointestinal Neuroendocrine Tumor G1

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

Kasajima A, Klöppel G
Pathologie (Heidelb) 2024 Nov;45(Suppl 1):51-55. Epub 2024 Oct 2 doi: 10.1007/s00292-024-01360-3. PMID: 39356330
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
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
Tatsuta T, Yoshimura T, Hasui K, Takasugi K, Sawaya M, Hanabata N, Shimoyama T, Kijima H, Fukuda S
Intern Med 2013;52(15):1697-701. Epub 2012 Mar 1 doi: 10.2169/internalmedicine.52.0083. PMID: 23903502
Ezziddin S, Opitz M, Attassi M, Biermann K, Sabet A, Guhlke S, Brockmann H, Willinek W, Wardelmann E, Biersack HJ, Ahmadzadehfar H
Eur J Nucl Med Mol Imaging 2011 Mar;38(3):459-66. Epub 2010 Sep 18 doi: 10.1007/s00259-010-1610-2. PMID: 20852858

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
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
Tatsuta T, Yoshimura T, Hasui K, Takasugi K, Sawaya M, Hanabata N, Shimoyama T, Kijima H, Fukuda S
Intern Med 2013;52(15):1697-701. Epub 2012 Mar 1 doi: 10.2169/internalmedicine.52.0083. PMID: 23903502

Therapy

Hirai R, Haruma K, Okada H, Itakura J, Mizuno M
Clin J Gastroenterol 2022 Apr;15(2):363-367. Epub 2022 Jan 4 doi: 10.1007/s12328-021-01581-6. PMID: 34982362
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
Ezziddin S, Opitz M, Attassi M, Biermann K, Sabet A, Guhlke S, Brockmann H, Willinek W, Wardelmann E, Biersack HJ, Ahmadzadehfar H
Eur J Nucl Med Mol Imaging 2011 Mar;38(3):459-66. Epub 2010 Sep 18 doi: 10.1007/s00259-010-1610-2. PMID: 20852858

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
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
Tatsuta T, Yoshimura T, Hasui K, Takasugi K, Sawaya M, Hanabata N, Shimoyama T, Kijima H, Fukuda S
Intern Med 2013;52(15):1697-701. Epub 2012 Mar 1 doi: 10.2169/internalmedicine.52.0083. PMID: 23903502

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
Sun H, Zhang S, Liu K, Zhou J, Wang X, Shen T, Wang X
Acta Radiol 2019 Nov;60(11):1394-1404. Epub 2019 Mar 26 doi: 10.1177/0284185119840212. PMID: 30913907
Tatsuta T, Yoshimura T, Hasui K, Takasugi K, Sawaya M, Hanabata N, Shimoyama T, Kijima H, Fukuda S
Intern Med 2013;52(15):1697-701. Epub 2012 Mar 1 doi: 10.2169/internalmedicine.52.0083. PMID: 23903502
Ezziddin S, Opitz M, Attassi M, Biermann K, Sabet A, Guhlke S, Brockmann H, Willinek W, Wardelmann E, Biersack HJ, Ahmadzadehfar H
Eur J Nucl Med Mol Imaging 2011 Mar;38(3):459-66. Epub 2010 Sep 18 doi: 10.1007/s00259-010-1610-2. PMID: 20852858

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