Abstract
We report a case of signet ring cell carcinoma of the ampulla of Vater. A 69-year old woman seen for vomiting was found in abdominal computed tomography and magnetic resonance imaging to have wall thickening of the second portion of the duodenum and marked dilation of the common bile duct and main pancreatic duct. Based on a preoperative diagnosis of carcinoma of the ampulla of Vater, we conducted pancreaticoduodenectomy with D2 lymph node dissection. Macroscopic findings of resected specimen revealed stenosis of the duodenal lumen around the ampulla of Vater with marked duodenal wall thickening, but without any erosion or ulceration of duodenum and bile duct. Histological studies showed signet ring cell carcinoma at the ampulla of Vater widely disseminated around the ampulla and infiltrating the pancreas and duodenum, together with infiltration beyond the serosa and lymph node involvement. After undergoing adjuvant chemotherapy with S-1 oral administration for one year, the woman has remained disease-free in the 3 years and 1 month since surgery. Signet ring cell carcinoma of the ampulla of Vater is rare and we review 26 cases in the Japanese and English literature.