Abstract： Objective To investigate the histological features,biological features,clinical treatment and prognosis of pancreatic acinar cell carcinoma.Methods A retrospective review of 10 patients with pancreatic acinar cell carcinoma treated in our hospital from 1999 to 2008 was conducted and the clinical features,imaging changes,pathologic feature,treatment course and follow-up data were collected.Results There were 9 men and 1 woman with a mean age of (62±8) years old.Tumors were located in the uncinate process in 1 patient,head of pancreas in 7,body and tail in 2.The median size of these tumors was 4.5 cm×4.7 cm;common bile duct and intrahepatic bile duct,pancreatic duct dilation was detected in 7 cases,and superior mesenteric vein was invaded in 2 cases.Of the 10 patients,8 received pancreaticoduodenectomy,among these 8 patients,3 had extended lymph node dissection,2 had portal vein resection and replacement;2received resection of pancreatic body and tail as well as splenectomy.Histologically,the size of these tumors were 4.0 cm×3.3 cm×3.4 cm.Macrescopically,duodenum was invaded in 5 patients,superior mesenteric vein was invaded in 2 patients and neural invasion was present in 7 cases.Lymph node metastasis was noted in 6 cases.Follow-up data was available in 9 patients and 1 patient was lost in follow-up.The survival ranged from 3 to 51 months with a median survival 18 months,and 9 patients died of tumor recurrence and metastasis after operation.Conclusions Pancreatic acinar cell carcinoma should be recognized as a distinct tumor entity and it may not be sensitive to radiotherapy or chemotherapy.The biological features of pancreatic acinar cell carcinoma should be investigated further.