BACKGROUND:Acinar cell carcinoma (ACC) is a rare malignancy of the pancreas arising from acinar cells.Unlike ductal adenocarcinoma,this tumor rarely presents with pancreatitis.METHODS:We present a case of ACC associated with chronic calcifying pancreatitis,and a review of the literature focusing on diagnosis and management.RESULTS:A 43-year-old man was proposed for Wirsungojejunal derivation for chronic pancreatitis.Histopathological examination of the tissue extracted revealed an ACC.Duodenopancreatectomy was performed.Six months postoperatively,the patient developed hepatic metastasis and was treated with gemcitabine as palliative chemotherapy.CONCLUSIONS:The clinical presentation of ACC of the pancreas is not specific and the tumor can be underdiagnosed when associated with chronic pancreatitis.Data regarding course,treatment,and prognosis of this tumor are generally lacking. BACKGROUND: Acinar cell carcinoma (ACC) is a rare malignancy of the pancreas arising from acinar cells. Unlike ductal adenocarcinoma, this tumor rarely presents with pancreatitis. METHODS: We present a case of ACC associated with chronic calcifying pancreatitis, and a review of the literature focusing on diagnosis and management .RESULTS: A 43-year-old man was proposed for Wirsungojejunal derivation for chronic pancreatitis. Histopathological examination of the tissue extracted revealed an ACC. Duodenopancreatectomy was performed. Six months postoperatively, the patient developed hepatic metastasis and was treated with gemcitabine as palliative chemotherapy. CONCLUSIONS: The clinical presentation of ACC of the pancreas is not specific and the tumor can be underdiagnosed when associated with chronic pancreatitis. Data regarding course, treatment, and prognosis of this tumor are generally lacking.