Published online Jun 15, 2016. doi: 10.4251/wjgo.v8.i6.509
Peer-review started: December 24, 2015
First decision: January 30, 2016
Revised: February 2, 2016
Accepted: March 14, 2016
Article in press: March 16, 2016
Published online: June 15, 2016
Core tip: Adenocarcinoma of small intestine (SBA) is a relatively rare malignancy with poor outcomes due to delayed diagnosis. Certain genetic and familial diseases are associated with increased risks for SBA. These include Familial adenomatous polyposis, lynch syndromes, juvenile polyposis syndrome, Peutz-Jeghers syndrome, Crohn’s disease and celiac disease. We discuss the clinical implications of this aggressive cancer focusing on the genetic and familial associations, signaling mechanisms and available diagnostic modalities for surveillance.