title: Hereditary Factors in Esophageal Adenocarcinoma.
abstract: BACKGROUND
  The vast majority of Barrett's esophagus (BE) and esophageal adenocarcinoma (EAC) cases are sporadic and caused by somatic mutations. However, over the last decades several families have been identified with clustering of EAC. Here, we review data from the published literature in order to address the current knowledge on familial EAC.
  
  
  CONCLUSIONS
  Although familial EAC comprises a relatively small group of patients, it is a clinically relevant category due to the poor prognosis of this type of cancer. Efforts should be made to identify specific genetic risk factors for familial EAC to enable identification of relatives at risk, since endoscopic surveillance can diagnose preneoplastic or early neoplastic lesions leading to early treatment, with improved outcome.
  
  
  CONCLUSIONS
  Although familial EAC comprises a relatively small group of patients, this is a clinically relevant category due to the poor prognosis. Efforts should be made to identify specific genetic risk factors for familial EAC in order to facilitate the identification of other family members with a predisposition for this type of cancer.
  
  
  CONCLUSIONS
  Approximately 7% of BE and EAC cases are considered familial. Age at diagnosis is generally lower for patients with familial EAC as compared to sporadic cases, while other known risk factors for EAC, such as male gender and Caucasian ethnicity, do not differ between the two groups. In several described families with clustering of EAC the pattern of inheritance seems to be consistent with a rare autosomal dominant genetic trait. However, some association has been found with (attenuated) familial adenomatous polyposis, mismatch repair deficiency and recently with the genes MSR1, ASCC1 and CTHRC1. Nevertheless, no specific genetic predisposition has yet been identified.
authors.affiliation: Department of Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands ; Department of Pathology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: Anna M J
authors.initials: AM
authors.lastname: van Nistelrooij
authors.affiliation: Department of Pathology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: Winand N M
authors.initials: WN
authors.lastname: Dinjens
authors.affiliation: Department of Clinical Genetics, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: Anja
authors.initials: A
authors.lastname: Wagner
authors.affiliation: Department of Gastroenterology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: Manon C W
authors.initials: MC
authors.lastname: Spaander
authors.affiliation: Department of Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: J Jan B
authors.initials: JJ
authors.lastname: van Lanschot
authors.affiliation: Department of Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
authors.forename: Bas P L
authors.initials: BP
authors.lastname: Wijnhoven
keywords: Barrett's esophagus; Esophageal adenocarcinoma; Hereditary factors