Abstract
Mixed neuroendocrine-non-neuroendocrine tumors (MiNEN) are a rare type of tumor formed by two components, a non-neuroendocrine component that is most often an adenocarcinoma and a neuroendocrine tumor, and each of these components must represent at least 30% of the tumor. The origin of this tumor on the ampulla of Vater or periampullary region is more infrequent. Usually, the lesions are highly aggressive and quickly metastasizing, and their biological behavior is dictated by the high grade of the neuroendocrine component. This is the first report of a patient with ampullary MiNEN treated employing a robotic pancreaticoduodenectomy. Although being submitted to aggressive treatment with complete surgical resection followed by systemic therapy, the patient developed early recurrence with hepatic metastatic disease, demonstrating the hostile nature of these tumors.