A Case of Adenoendocrine Cell Carcinoma in the Papilla of Vater

A 77-years-old-man admitted for body weight loss was found to have an elevated ulcerative tumor located in the papilla of Vater. Histological examination of a biopseid specimen showed undifferentiated adenocarcinoma. We conducted pancreaticoduodenostomy with regional lymph node dissection. The resected specimen showed an intra-ampullary tumor within the oddi muscle layer. Histopathologically, the tumor consisted of solid nests of atypical argyrophilic cells, with adenocarcinoma replacing mucosa. Immunohistochemical analysis showed positive staining for NSE and chromogranin A. The final diagnosis was adenoendocrine cell carcinoma of the papilla of Vater. Adenoendocrine cell carcinoma may originate from adenocarcinoma characterized by high-grade malignancy and yielding a dismal prognosis. The patient died from multiple liver metastasis 14 months after surgery. To the best of our knowledge, this is the 14th case of adenoendocrine cell carcinoma of the papilla of Vater reported in Japan, indicating the rarity of such case so.