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Mixed Neuroendocrine Non-Neuroendocrine Neoplasm of the Ampulla of Vater: Report of a Rare Location

Year: 2025

Type: article

Abstract: Background: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) are rare tumors of the gastrointestinal tract with neuroendocrine and non-neuroendocrine components. Ampullary MiNENs are extremely rare, with few cases reported to date. Case Report: A 41-year-old male was diagnosed incidentally with a dilated common bile duct and intrahepatic biliary radicles while being evaluated for right ureteric calculi. Contrast-enhanced computed tomography scan of the abdomen showed a mass in the ampullary region with a positive double duct sign. Side-viewing endoscopy indicated an ampullary growth, and biopsy confirmed adenocarcinoma. The patient underwent total robotic pancreatoduodenectomy with an uneventful postoperative course. His final histopathologic examination revealed a tumor with 2 components, each of which accounted for at least 30% of the tumor: a neuroendocrine tumor and an adenocarcinoma with signet ring cells. The patient received adjuvant chemotherapy and at 1-year follow-up showed no evidence of recurrence. Conclusion: Ampullary MiNENs are rare composite gastroenteropancreatic tumors characterized by histologic heterogeneity; they can be effectively treated with robotic pancreatoduodenectomy. The more aggressive component of the MiNEN should be considered when determining an adjuvant therapy.

Source: Ochsner Journal

DOI: 10.31486/toj.25.0030

Authors: Ankit Rai, Vaibhav Kumar Varshney, Peeyush Varshney, Lokesh Agarwal, Meenakshi Rao, Ayushi Agarwal

Institution: All India Institute of Medical Sciences Jodhpur

Language: English

Topic: Neuroendocrine Tumor Research Advances

Subfield: Epidemiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: diamond

Funder: Ochsner Health