Mixed neuroendocrine non-neuroendocrine neoplasm of the ampulla of Vater: Case Report

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Introduction: Mixed neuroendocrine non-neuroendocrine neoplasm (MiNEN) is defined as a tumor composed of both neuroendocrine and non-neuroendocrine components. MiNEN of the ampulla of Vater is a rare neuroendocrine tumor of the gastrointestinal tract.

Aim(s): To present the clinical case of a man with MiNEN of the ampulla of Vater.

Materials and methods: A 64-year-old man presented in another hospital with dark urine and jaundice. Contrast-enhanced computed tomography showed enlargement of the intra and extrahepatic bile ducts. Magnetic resonance cholangiopancreatography revealed a neoplastic lesion at the end of the common bile duct. A pancreaticoduodenectomy was performed. Histopathological examination showed a complex lesion with two tumoral components that were an adenocarcinoma with focal areas of squamous-cell carcinoma and a neuroendocrine component positive for neuroendocrine markers, with a Ki-67 labelling index between 30 and 40%. About 17 months after the surgery, a magnetic resonance showed liver and lung metastases. A lung biopsy disclosed metastatic adenocarcinoma with focal squamous-cell carcinoma. The samples of primary tumor were reviewed, and it was found that both components of the neoplasm represented >30% of the tumor with a Ki-67 index of 50%. The diagnosis of MiNEN of the ampulla of Vater was confirmed. The patient was proposed to chemotherapy but till now refused the treatment. Three years after the surgery, the patient remains asymptomatic.

Conference: 18th Annual ENETS Concerence (2021)

Presenting Author: Caetano A

Authors: Caetano A, Sousa M, Elvas A, Monteiro A, Couto J,

Keywords: mixed neuroendocrine non-neuroendocrine neoplasm, MiNEN, ampulla of Vater, neuroendocrine tumor,