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Small intestine carcinoid

MedGen UID:
Concept ID:
Finding; Neoplastic Process
HPO: HP:0006722

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Small intestine carcinoid

Conditions with this feature

Familial adenomatous polyposis 1
MedGen UID:
Concept ID:
Disease or Syndrome
APC-associated polyposis conditions include (classic or attenuated) familial adenomatous polyposis (FAP) and gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). FAP is a colorectal cancer (CRC) predisposition syndrome that can manifest in either classic or attenuated form. Classic FAP is characterized by hundreds to thousands of adenomatous colonic polyps, beginning on average at age 16 years (range 7-36 years). For those with the classic form of FAP, 95% of individuals have polyps by age 35 years; CRC is inevitable without colectomy. The mean age of CRC diagnosis in untreated individuals is 39 years (range 34-43 years). The attenuated form is characterized by multiple colonic polyps (average of 30), more proximally located polyps, and a diagnosis of CRC at a later age than in classic FAP. For those with an attenuated form, there is a 70% lifetime risk of CRC and the mean age of diagnosis is 50-55 years. Extracolonic manifestations are variably present and include polyps of the stomach and duodenum, osteomas, dental abnormalities, congenital hypertrophy of the retinal pigment epithelium (CHRPE), benign cutaneous lesions, desmoid tumors, adrenal masses, and other associated cancers. GAPPS is characterized by proximal gastric polyposis, increased risk of gastric adenocarcinoma, and no duodenal or colonic involvement in most individuals reported.

Professional guidelines


Lim JY, Pommier RF
Front Endocrinol (Lausanne) 2021;12:622693. Epub 2021 Feb 26 doi: 10.3389/fendo.2021.622693. PMID: 33732215Free PMC Article
Strosberg JR, Halfdanarson TR, Bellizzi AM, Chan JA, Dillon JS, Heaney AP, Kunz PL, O'Dorisio TM, Salem R, Segelov E, Howe JR, Pommier RF, Brendtro K, Bashir MA, Singh S, Soulen MC, Tang L, Zacks JS, Yao JC, Bergsland EK
Pancreas 2017 Jul;46(6):707-714. doi: 10.1097/MPA.0000000000000850. PMID: 28609356Free PMC Article
Liu EH, Solorzano CC, Katznelson L, Vinik AI, Wong R, Randolph G
Endocr Pract 2015 May;21(5):534-545. doi: 10.4158/EP14464.DSC. PMID: 25962092Free PMC Article

Recent clinical studies


Abou Saleh M, Mansoor E, Anindo M, Isenberg G
Dig Dis Sci 2019 May;64(5):1328-1334. Epub 2018 Dec 5 doi: 10.1007/s10620-018-5402-z. PMID: 30519851
Housset C, Chrétien Y, Debray D, Chignard N
Compr Physiol 2016 Jun 13;6(3):1549-77. doi: 10.1002/cphy.c150050. PMID: 27347902
Nogueira L, Freedman ND, Engels EA, Warren JL, Castro F, Koshiol J
Am J Epidemiol 2014 Mar 15;179(6):731-9. Epub 2014 Jan 26 doi: 10.1093/aje/kwt322. PMID: 24470530Free PMC Article
Weiss NS, Yang CP
J Natl Cancer Inst 1987 Apr;78(4):653-6. PMID: 3470541


Ulbright TM, Young RH
Am J Surg Pathol 2008 Nov;32(11):1683-93. doi: 10.1097/PAS.0b013e3181788516. PMID: 18769334


Collet e Silva F
Rev Paul Med 1993 Mar-Apr;111(2):378-80. PMID: 8284583

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