Upper gastrointestinal cancer predisposition syndromes.

Overview

Malignancies of the upper gastrointestinal tract form a heterogeneous group of cancers characterized by unique epidemiology and biology. Despite these differences, survival for advanced disease remains poor across the panel of diseases, from cancers of the esophagus, stomach, pancreas, and, until recently, even gastrointestinal stromal tumors. Genetic predisposition syndromes associated with these diseases comprise an emerging subset of these diseases that may provide valuable information on cause and etiology. They may provide insight into molecular drivers for the disease, or disease subtypes, and also insights into novel gene/environment interactions. This review summarizes the current understanding of genetic predisposition syndromes of cancers of the upper gastrointestinal tract.