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

Ankit Rai, Vaibhav Kumar Varshney, Peeyush Varshney, Lokesh Agarwal, Meenakshi Rao and Ayushi Agarwal
Ochsner Journal June 2025, DOI: https://doi.org/10.31486/toj.25.0030

Article Figures & Data

Figures

  • Figure 1.
    Figure 1.

    Contrast-enhanced computed tomography (A) axial reformatted image in pancreatic phase shows an ill-defined mass in the ampullary region with peripheral rim enhancement (arrow). (B) Coronal reformatted image in pancreatic phase shows a smooth bulge into the medial wall of the second part of the duodenum (arrow), as well as cutoff of the distal common bile duct (arrowhead) and pancreatic duct (asterisk) with upstream dilatation (the double duct sign). (C) Axial and (D) coronal reformatted images in portal venous phase show progressive enhancement of the mass (arrows), with the mass appearing iso-hyperenhancing to the pancreatic parenchyma (asterisk in image D). (E) Axial image in portal venous phase shows bilobar intrahepatic biliary dilatation. No focal lesions were noted in the liver parenchyma. (F) Coronal reformatted image in portal venous phase shows heterogeneously enhancing subcentimetric, periportal, peripancreatic, and mesenteric lymph nodes (arrows).

  • Figure 2.
    Figure 2.

    Intraoperative images show (A) port placement for the robotic pancreatoduodenectomy, (B) stomach transection using the robotic SureForm stapler (Intuitive Surgical, Inc), (C) duct-to-mucosa pancreaticojejunal anastomosis, (D) hepaticojejunal anastomosis, (E) pancreatoduodenectomy surgical specimen, and (F) postoperative abdomen with minimal scars.

  • Figure 3.
    Figure 3.

    (A) Cut section of the gross pancreatoduodenectomy specimen shows the ampullary growth circled in red. (B) Low-power view of the periampullary tumor shows the neuroendocrine tumor component (composed of well-formed nests of monomorphic cells) in the upper left half of the image and the adenocarcinoma (with signet ring cell components) in the lower right corner of the image (hematoxylin and eosin [H&E] stain, magnification ×100). (C) Low-power view shows the adenocarcinoma with signet ring cells in the duodenal mucosa, with entrapped benign duodenal glands in between (H&E stain, magnification ×100). (D) The duodenal wall also shows a well-differentiated neuroendocrine tumor composed of nests of monomorphic cells in the muscle layer on the right side of the image (H&E stain, magnification ×100). (E) Chromogranin A immunohistochemistry demonstrates chromogranin A positivity in the neuroendocrine tumor component. (F) Immunohistochemistry for the pancytokeratin cocktail (AE1/AE3) highlights the infiltrating signet ring cells in the duodenal mucosa.

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