[A Case of Pancreatic Acinar Cell Carcinoma with Invasive Ductal Carcinoma and Neuroendocrine Tumor]

A 50-year-old female presented with abdominal pain. Upper gastrointestinal endoscopy revealed a 30 mm ulcerative lesion extending from the duodenal bulb to the descending portion, and biopsy confirmed poorly differentiated adenocarcinoma. Abdominal contrast-enhanced CT scan showed an hypovascular tumor in the pancreatic head with suspected invasion into the duodenum, along with enlarged #8 lymph node. PET-CT revealed abnormal uptake in the pancreatic head and #8 lymph node. She underwent robotic pancreaticoduodenectomy for a diagnosis of pancreatic head cancer with lymph node metastasis. Histopathological examination revealed proliferation of atypical cells in acinar, trabecular, ribbon-like anastomosing, and tubular structures. Immunohistochemistry showed positivity for Bcl10(60%), INSM1(20%), Ki-67 index of 45%, and coexistence of invasive ductal adenocarcinoma(20%). Based on these findings, the tumor was diagnosed as predominantly acinar cell carcinoma with invasive ductal adenocarcinoma and neuroendocrine tumor. Lymph node metastasis was positive showing Bcl10-positive acinar cell carcinoma. Four months postoperatively, recurrence was detected in the para-aortic lymph nodes, confirmed by biopsy to be acinar cell carcinoma. Chemotherapy was initiated but discontinued due to tumor progression leading to best supportive care.