INTRODUCTION: The risk of periampullary neoplasia in patients with familial adenomatous polyposis (FAP) is significantly increased compared to the general population. PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: 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.
INTRODUCTION: The risk of periampullary neoplasia in patients with familial adenomatous polyposis (FAP) is significantly increased compared to the general population. PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: 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.
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