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Carcinoid tumor of intestine

MedGen UID:
138099
•Concept ID:
C0349535
•
Neoplastic Process
Synonyms: carcinoid tumors, intestina; Carcinoid tumors, intestinal; Intestinal neuroendocrine tumor G1
 
Monarch Initiative: MONDO:0021533
OMIM®: 114900

Definition

A well differentiated, low grade neuroendocrine neoplasm that arises from the small or large 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]

Clinical features

From HPO
Intestinal carcinoid
MedGen UID:
870540
•Concept ID:
C4024988
•
Neoplastic Process
See: Feature record | Search on this feature

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
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
Principles of diagnosis and management of neuroendocrine tumours.
Raphael MJ, Chan DL, Law C, Singh S
CMAJ 2017 Mar 13;189(10):E398-E404. doi: 10.1503/cmaj.160771. PMID: 28385820Free PMC Article

Recent clinical studies

Etiology

Endometriosis and its correlation with carcinoid tumor of the appendix: a systematic review.
Villaescusa M, Andres MP, Amaral AC, Barbosa RN, Abrão MS
Minerva Obstet Gynecol 2021 Oct;73(5):606-613. Epub 2021 Apr 27 doi: 10.23736/S2724-606X.21.04792-4. PMID: 33904692
The Problem of Appendiceal Carcinoids.
Galanopoulos M, Toumpanakis C
Endocrinol Metab Clin North Am 2018 Sep;47(3):661-669. Epub 2018 Jul 13 doi: 10.1016/j.ecl.2018.04.004. PMID: 30098722
Periampullary carcinoid tumor.
Fukatsu H, Kawamoto H, Fujii M, Tsutsumi K, Kato H, Hirao K, Kurihara N, Okamoto Y, Ogawa T, Ishida E, Okada H, Sakaguchi K
Endoscopy 2007 Feb;39 Suppl 1:E49-50. Epub 2007 Feb 7 doi: 10.1055/s-2006-945068. PMID: 17285506
Small-bowel tumors.
Minardi AJ Jr, Zibari GB, Aultman DF, McMillan RW, McDonald JC
J Am Coll Surg 1998 Jun;186(6):664-8. doi: 10.1016/s1072-7515(98)00092-1. PMID: 9632155
Unusual causes of mesenteric ischemia.
Krupski WC, Selzman CH, Whitehill TA
Surg Clin North Am 1997 Apr;77(2):471-502. doi: 10.1016/s0039-6109(05)70562-4. PMID: 9146726

Diagnosis

Goblet cell adenocarcinoma of the appendix: an update and practical approach to diagnosis and grading.
Bell PD, Pai RK
Hum Pathol 2023 Feb;132:183-196. Epub 2022 Jun 9 doi: 10.1016/j.humpath.2022.06.002. PMID: 35691370
The Incidental Goblet Cell Adenocarcinoma.
Nugent TS, Kelly ME
Dis Colon Rectum 2022 Nov 1;65(11):1301-1304. Epub 2022 Aug 25 doi: 10.1097/DCR.0000000000002595. PMID: 36228498
The hairpin turn.
Bailey R, LeGout JD
Abdom Radiol (NY) 2020 Apr;45(4):1218-1219. doi: 10.1007/s00261-020-02422-9. PMID: 32008113
Carcinoid tumors.
Robertson RG, Geiger WJ, Davis NB
Am Fam Physician 2006 Aug 1;74(3):429-34. PMID: 16913162
Carcinoid syndrome.
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912

Therapy

Somatostatin Analogs in Clinical Practice: a Review.
Gomes-Porras M, Cárdenas-Salas J, Álvarez-Escolá C
Int J Mol Sci 2020 Feb 29;21(5) doi: 10.3390/ijms21051682. PMID: 32121432Free PMC Article
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
Gastroenteropancreatic Neuroendocrine Tumors.
Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Carcinoid syndrome.
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912
Abdominal surgery.
Welch CE
N Engl J Med 1969 Feb 20;280(8):419-25 contd. doi: 10.1056/NEJM196902202800805. PMID: 4883944

Prognosis

Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Gastroenteropancreatic Neuroendocrine Tumors.
Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Periampullary carcinoid tumor.
Fukatsu H, Kawamoto H, Fujii M, Tsutsumi K, Kato H, Hirao K, Kurihara N, Okamoto Y, Ogawa T, Ishida E, Okada H, Sakaguchi K
Endoscopy 2007 Feb;39 Suppl 1:E49-50. Epub 2007 Feb 7 doi: 10.1055/s-2006-945068. PMID: 17285506
Carcinoid tumors.
Robertson RG, Geiger WJ, Davis NB
Am Fam Physician 2006 Aug 1;74(3):429-34. PMID: 16913162
Carcinoid syndrome.
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912

Clinical prediction guides

Clinical Spectrum and Endoscopic Treatment of Gastrointestinal Carcinoid Tumour.
Xue L, Cai Y, Chen W, Chen S, Xue P
J Coll Physicians Surg Pak 2022 Oct;32(10):1330-1333. doi: 10.29271/jcpsp.2022.10.1330. PMID: 36205280
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Cardiac Metastases of Small-Bowel Carcinoid(Added Value of (18)F-Fluorodihydroxyphenylalanine Positron Emission Tomography Combined to Magnetic Resonance Imaging).
El Ghannudi S, Ben Abdelghani M, Germain P, Blondet C, Romain B, Imperiale A
Circ Cardiovasc Imaging 2019 Feb;12(2):e008405. doi: 10.1161/CIRCIMAGING.118.008405. PMID: 30744393
Gastroenteropancreatic Neuroendocrine Tumors.
Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Expression of growth factors and their receptors in neuroendocrine gut and pancreatic tumors, and prognostic factors for survival.
Oberg K
Ann N Y Acad Sci 1994 Sep 15;733:46-55. doi: 10.1111/j.1749-6632.1994.tb17255.x. PMID: 7978895

Recent systematic reviews

Management Strategies and Outcomes for Small Intestinal Neuroendocrine Tumours with Involvement of the Superior Mesenteric Vessels: A Systematic Review.
Kmiotek E, Lakda S, Borakati A, Ogunbiyi O, Mandair D, Caplin M, Toumpanakis C, Mirnezami R
Curr Oncol 2023 Oct 18;30(10):9192-9204. doi: 10.3390/curroncol30100664. PMID: 37887564Free PMC Article
Endometriosis and its correlation with carcinoid tumor of the appendix: a systematic review.
Villaescusa M, Andres MP, Amaral AC, Barbosa RN, Abrão MS
Minerva Obstet Gynecol 2021 Oct;73(5):606-613. Epub 2021 Apr 27 doi: 10.23736/S2724-606X.21.04792-4. PMID: 33904692
Rectal carcinoids: a systematic review.
McDermott FD, Heeney A, Courtney D, Mohan H, Winter D
Surg Endosc 2014 Jul;28(7):2020-6. Epub 2014 Mar 1 doi: 10.1007/s00464-014-3430-0. PMID: 24584484
Systematic review of resection of primary midgut carcinoid tumour in patients with unresectable liver metastases.
Capurso G, Rinzivillo M, Bettini R, Boninsegna L, Delle Fave G, Falconi M
Br J Surg 2012 Nov;99(11):1480-6. Epub 2012 Sep 13 doi: 10.1002/bjs.8842. PMID: 22972490
Efficacy and safety of endoscopic submucosal dissection for colorectal neoplasia: a systematic review.
Repici A, Hassan C, De Paula Pessoa D, Pagano N, Arezzo A, Zullo A, Lorenzetti R, Marmo R
Endoscopy 2012 Feb;44(2):137-50. Epub 2012 Jan 23 doi: 10.1055/s-0031-1291448. PMID: 22271024

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