A primary extra-ampullary duodenal neuroendocrine carcinoma was found in a 40-year-old man who presented with upper abdominal pain and weight loss. Duodenoscopy and hypotonic duodenography revealed a protruding fungating mass with luminal occlusion at the third part of the duodenum (D3). Although the metastatic work-up was normal, the tumor was inoperable intraoperatively, hence a palliative bypass was carried out followed by chemotherapy with 5-fluorouracil and leucovorin.
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