Two cases of adenoendocrine cell carcinoma in the papilla of Vater

Abstract

We here report two cases of adenoendocrine cell carcinoma occurring in the papilla of Vater. Both patients underwent PPPD. Microscopically, tumor cells proliferated in solid nests together with adenocarcinoma component. Immunohistochemical analysis showed positive staining for neuroendocrine markers. Case 1: A 55-yearold man was admitted with a diagnosis of acute pancreatitis. The tumor was pT3, pN2, H0, P0, M (-), fStageIVa He died of multiple liver metastases 5 months after surgery. Case 2: A 72-year-old man was admitted with a diagnosis of acute cholangitis and obstructive jaundice. The tumor was pT3, pN1, H0, P0, M (-), fStageIII. He has not shown any evidence of recurrence 1 year after surgery. Only 5 cases of adenoendocrine carcinoma in the papilla of Vater have been reported in Japan; they have shown extremely poor prognosis with early metastases to either liver or lymph nodes. It is therefore suggested that effective postoperative adjuvant therapy in addition to radical surgery would be necessary.