A 75-year-old woman underwent upper endoscopy and was revealed tumorous lesions at the duodenal papilla. Preoperative biopsy showed well-differentiated tubular adenocarcinoma histopathologically, she then underwent surgical operation. Histological examination of the surgical specimens revealed coexistence of neuroendocrine carcinoma (NEC), small cell type, and adenocarcinoma components of the duodenal ampullary resion.
The NEC component formed a polypoid lesion in the distal bile duct, while the adenocarcinoma component exhibited intramucosal spreding in the pancreatic duct. Therefore, the tumorous lesion was histologically diagnosed as primary mixed adenoneuroendocrine carcinoma (MANEC) of the papilla. Multiple hepatic metastases were detected 2 months after surgery, and the patient died 8 months after surgery. The prognosis of MANEC is poor, and the standard surgical procedure and chemotherapy regimen for MANEC have not been determined. Herein, we report a case of MANEC, along with a review of the literature.
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