Pancreatic Acinar Cell Carcinoma Presenting with Diffuse Pancreatic Enlargement

Pancreatic acinar cell carcinoma (PACC) is a rare tumor of the exocrine pancreas. It accounts for only 1% of all pancreatic malignancies. A 39-year woman sought treatment for repeated abdominal pain and jaundice. The computed tomography (CT) and magnetic resonance imaging (MRI) suggested diffuse enlargement of the pancreas. 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT showed diffuse enlargement of the pancreas and increased glucose uptake. The endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) biopsy of the pancreas was performed, and the case was finally diagnosed as PACC. After the sixth course of chemotherapy with gemcitabine and albumin paclitaxel, a CT scan showed significant shrinkage of the pancreas. PACC very rarely presents with diffuse enlargement of the pancreas. This case illustrates a rare presentation of PACC with diffuse pancreatic enlargement, which was effectively treated with gemcitabine and albumin paclitaxel chemotherapy. Key Words: Pancreatic acinar cell carcinoma, Positron emission tomography/computed tomography, Chemotherapy.