Abstract
A 46-year-old man admitted to our hospital with jaundice was found to have a tumor of the ampulla of Vater. A preoperative tumor biopsy showed nested, trabecular cells with an organoid appearance. Immunochemical findings showed that these cells were positive for chromogranin A, synaptophysin, and CD56. The Ki-67 index was over 90%. Ampullary neuroendocrine carcinoma was diagnosed, and the patient was treated by pancreaticoduodenectomy. Postoperative histological assessment revealed large cell neuroendocrine carcinoma (LCNEC) of the ampulla of Vater. The patient declined adjuvant chemotherapy, but he had to start chemotherapy with cisplatin and etoposide because multiple liver and lymph node metastases occurred three months after the surgery. After four courses of the chemotherapy, PD was assessed. His chemotherapy was thus changed to gemcitabine + S-1 (GS) as a second line. However, he was hospitalized because of cancerous peritonitis during four courses of GS, and he died of cancer 11 months after the surgery. LCNEC of the ampulla of Vater is extremely rare among ampullary tumors in the world, and its prognosis is poor.
