Large Cell Neuroendocrine Tumors (LCNEC) in the ampulla of Vater are extremely rare. This
report addresses a case of concurrent LCNEC and adenocarcinoma in the ampulla of Vater. A
60-year-old male patient experienced fever, body chills and jaundice. He had a periampullary
ulcerative lesion and underwent radical pancreaticoduodenectomy. Histopathologically, the
tumor consisted of an LCNEC component and an adenocarcinoma component. Simultaneous
LCNEC and adenocarcinoma has been reported in a few cases. Our patient had a coexisting
LCNEC and an adenocarcinoma of the ampulla of Vater. We also present a review of the
literature.