Rare case of acinar cell carcinoma with multiple lesions in the pancreas

We present the first case of pancreatic acinar cell carcinoma (PACC) with multiple lesions. A 55‐year‐old man with a pancretic tail mass on abdominal computed tomography (CT) was admitted to our hospital. Endoscopic ultrasound (EUS) showed a hypoechoic mass, and EUS‐guided fine‐needle aspiration (EUS‐FNA) revealed the mass to be PACC. The patient underwent distal pancreatectomy, and two masses were identified in the pancreatic tail and body. Histologically, both masses had tumor cells similar to acinar cells and were positive for BCL‐10. The patient was thus diagnosed with synchronous PACC. Ten months after the surgery, abdominal CT revealed a mass in the remnant pancreas. EUS showed a hypoechoic mass, and EUS‐FNA determined it to be PACC. The patient underwent total remnant pancreatectomy. The histological imaging results were similar to those of the first resection. Finally, the patient was diagnosed with synchronous and metachronous PACC. The possibility of multiple occurrences in the pancreas should be considered with PACC.