Sarcomatoid Adenocarcinoma of the Ampulla of Vater

Sarcomatoid adenocarcinoma of ampulla of Vater is an extremely rare malignant neoplasm that displays both carcinomatous and sarcomatous component. A 58-year-old woman was admitted to our hospital under the suspicion of an ampulla of Vater cancer. Abdominal computed tomography and endoscopy demonstrated a bulging of ampulla and the biopsy specimen revealed an adenocarcinoma, well differentiated in the background of tubulovillous adenoma. So we performed the pylorus preserving pancreaticoduodenectomy. At postoperative biopsy, the tumor was composed of adenocarcinoma component and sarcomatoid component. Thus, a diagnosis of sarcomatoid adenocarcinoma of ampulla of Vater could be made. Here, we present a case of sarcomatoid adenocarcinoma of ampulla of Vater.