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Mixed malignant ampullary tumours displaying adenomatous and neuroendocrine features are infrequent, with only nine reported cases, and almost all of them are composed of intestinal-type adenocarcinoma and well-differentiated neuroendocrine/carcinoid tumour.1
An 81-year-old man, with personal history of arterial hypertension, hypercholesterolaemia, type 2 diabetes mellitus and a previous appendectomy, presented with abdominal pain, fever and obstructive jaundice of 4-day duration. Physical examination was unremarkable but blood analysis showed an elevated alkaline phosphatase of 189 U/l (normal 40–130 U/l), and an elevated γ-glutamyltransferase of 255 U/l (normal 11–49 U/l). Aminotransferases were slightly elevated. Abdominal ultrasonography revealed dilated gallbladder and extrahepatic bile ducts. A cholangioresonance showed an exophytic mass in the ampulla of Vater, while an abdominal CT indicated a nodular lesion of 2 cm in the papillary region, suggesting ampullary adenocarcinoma. An endoscopic retrograde cholangiopancreatography and sphincterotomy were performed, and cytology and endoscopic biopsy were taken, revealing malignant cells, consistent with adenocarcinoma. Whipple resection was eventually performed.
Grossly, the pancreaticoduodenectomy specimen showed an exophytic lesion of 2.7×1.6 cm obstructing the ampulla of Vater. A piece of this exophytic tumour was snap frozen in liquid nitrogen before fixation. H&E-stained sections of the entire lesion revealed an intestinal-type papillary tumour resembling a colonic villous adenoma, with cigar-shaped nuclei and some goblet cells, but with clear malignant features such as high nuclear atypia, high number of mitotic and apoptotic figures, and invasion in the lamina propria of the ampulla, with desmoplastic reaction. Some rests of peripheral adenoma could be seen. In some small areas this infiltrative intestinal-type …
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