title: FAP related periampullary adenocarcinoma.
abstract: BACKGROUND
  The risk of periampullary neoplasia in patients with familial adenomatous polyposis (FAP) is significantly increased compared to the general population.
  
  
  METHODS
  We herein report the case of a 47-year-old woman with classic familial adenomatous polyposis with a history of total proctocolectomy for FAP who presented with an ulcerous ampullary lesion 8 years after primary colorectal surgery. Interestingly, the patient had not enrolled to optimal postoperative upper endoscopy follow-up. The patient underwent a Whipple procedure. Histology demonstrated a T2N0 ampullary adenocarcinoma.
  
  
  CONCLUSIONS
  Periampullary disease in patients with familial adenomatous polyposis occurs increasingly, especially in the subset of patients without proper endoscopic follow-up. Current recommendations concerning upper endoscopy and appropriate management are herein discussed; the importance of optimal postoperative endoscopy after total proctocolectomy in the FAP setting is discussed.
  
  
  CONCLUSIONS
  Periampullary cancer carries a significant risk in patients with FAP and proper endoscopic follow-up should be applied in this special patient group in order to manage ampullary manifestations of the disease in a timely manner.
authors.affiliation: 2nd Department of Propedeutic Surgery, Faculty of Medicine, Athens University, "Laiko" General Hospital, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Mantas
authors.forename: Petros
authors.initials: P
authors.lastname: Charalampoudis
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Nikiteas
keywords: Ampullary adenocarcinoma; Endoscopy; Familial adenomatous polyposis; Total proctocolectomy