ABSTRACT
Pancreatic acinar cell carcinoma (ACC) is a rare neoplasm accounting less than 1% of malignant pancreatic tumors. A 47-year-old male patient visited the emergency room with epigastric pain. Computed tomography or magnetic resonance imaging revealed a 4.7-cm heterogeneously enhanced solid and cystic mass with internal necrosis located in the head of the pancreas. Radiological diagnosis was borderline malignancy such as neuroendocrine tumor or solid pseudopapillary neoplasm. Two months later, the necrotic mass in the pancreas head had grown up to 11 cm, compressing the duodenum, superior mesenteric vein, and proximal transverse colon. Pylorus preserving pancreatoduodenectomy with segmental resection of transverse colon was performed. Histopathological examination revealed that the tumor was pancreatic ACC. The patient recovered without any complication and was doing well without recurrence for 12 months after surgery.