case_images: PMC8565687_01
case_images: CECT of the abdomen, venous phase: sagittal (A), axial (B), coronal (C) views, showing duodenal wall thickening both above and beyond papilla (white arrowheads) with nodular aspect at para-ampullary level (*); biliary tree is mildly dilated; adenopathies (arrows) and fat stranding around them as well as peri-duodenal are also present. CECT: Contrast-enhanced computed tomography
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case_images: Afterwards, an abdominal contrast-enhanced computed tomography (CECT) scan was performed, describing circumferential wall thickening of the second segment of the duodenum up to the inferior duodenal flexure, para-ampullary and papillary nodular aspect with non-homogenous contrast enhancement, irregular margins, with no separation limit from the pancreatic parenchyma (Figure 1).
case_images: CECT of the abdomen and thorax, venous phase: abdomen axial (A), axial thorax, chest window (B) and lung window (C) showing single liver metastasis (black arrowhead), mediastinal and interlobar adenopathies (arrows), lung metastasis at the lungs base (white arrowheads).
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case_images: The CECT scan also identified a nodular hepatic lesion, enlarged abdominal lymph glands and nodular lesions in the inferior pulmonary lobes, with associated hilum and mediastinal adenopathies (confirmed by a subsequent thoracic CECT) (Figure 2).
case_images: Irregular hypertrophic duodenal mucosal folds suggestive for tumoral infiltration - endoscopic view
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case_images: Upper gastrointestinal endoscopy (UGE) described irregular hypertrophic duodenal mucosal folds suggestive for tumor infiltration (Figure 3) and biopsies were performed.
case_images: Duodenal large-cell neuroendocrine carcinoma: A) Isles of large, polygonal tumor cells in lamina propria and submucosa; numerous tumor emboli in vascular spaces (PAS, x100); B) Tumor cells are positive for Cytokeratin 7 (IHC, anti-CK7 Ab, x100); C) Synaptophysin is positive in tumor cells and normal enterochromaphin duodenal cells (arrow) (IHC, anti-Synaptophysin Ab, x100); D) Ki-67 positive in 80% of tumor cells and in proliferative compartment of duodenal crypts (arrow) (IHC, anti-Ki-67 Ab, x100).
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case_images: Histological examination using routine technique showed isles of large, polygonal tumor cells in lamina propria and submucosa, and numerous tumor emboli in vascular spaces (Figure 4.A). Immunohistochemical (IHC) tests were positive for cytokeratin CK7 (Figure 4.B) and synaptophysin in the tumor cells (Figure 4.C), and negative for chromogranin; Ki-67 proliferation index was 80% (Figure 4.D).
case_images: Internal-external percutaneous biliary drainage, left side approach
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case_images: Internal-external biliary drainage was placed percutaneously by left hepatic lobe approach (Figure 5), with good immediate evolution and fully functional drainage afterwards.
case_images: CECT of the abdomen and thorax 6 months later: axial lung upper regions (A), axial chest window at the base of the lungs (B), axial abdomen, liver (C). Lung metastasis are present also in upper lungs, increased size of mediastinal adenophaties, and multiple liver metastasis; functional percutaneous internal-external biliary drainage (black arrowhead) - aerobilia (white arrowhead)
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case_images: Unfortunately, tumor progression was recorded, as showed by a recent thoraco-abdominal CECT examination (Figure 6).