2016 Volume 31 Issue 4 Pages 688-696
We report a rare case of an α-fetoprotein (AFP) producing mixed acinar-neuroendocrine carcinoma of the pancreas. A 52-year-old male visited our hospital because of a tumor in the pancreatic tail. Abdominal contrast enhanced computed tomography revealed a hypovascular, well-marginated tumor 55mm in diameter. Laboratory findings showed normal CEA and CA19-9, but AFP was elevated at 65.9ng/ml and AFP-L3 was 87.1%. With a diagnosis of acinar cell carcinoma of the pancreatic tail, distal pancreatectomy was performed. Histopathological findings of the tumor revealed acinar structure with eosinophilic cytoplasm, and trabecular and alveolar structure. Immunohistochemical examination was positive for trypsin and AFP, moreover, positive for synaptophysin and chromogranin A. Trypsin and synaptophysin were positive in more than 30% of the tumor. The histological diagnosis was AFP producing mixed acinar-neuroendocrine carcinoma of the pancreas. In the Japanese literature, mixed acinar-neuroendocrine carcinoma of the pancreas is rare. We also summarize 32 cases with this rare disease.