Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater: Report of a case

Abstract

A 72-year-old woman, presenting with complaints of appetite loss and jaundice, was admitted to our hospital. CT revealed a well-enhanced tumor with a diameter of 20mm in the ampullary region. EUS showed a well-defined hypoechoic tumor. Biopsy identified small round tumor cells, positive for synaptophysin and strongly positive for Ki67, leading to a diagnosis of neuroendocrine carcinoma (NEC). Subsequently, a pylorus-preserving pancreaticoduodenectomy was performed. The pathological examination revealed a Ki67 index of 100%, leading to the diagnosis of T2N1M0. Adjuvant chemotherapy was not administered. After 2 years and 8 months postoperatively, multiple liver metastases, portal vein tumor thrombus, and peritoneal dissemination were detected. Carboplatin plus etoposide therapy was initiated, resulting in significant tumor reduction after one cycle. However, Grade 4 neutropenia and febrile neutropenia occurred, leading to discontinuation of chemotherapy. The patient passed away 2 years and 11 months postoperatively. Additional case accumulation is necessary to determine the optimal treatment for biliary NEC.