尽管胰腺80%由腺泡细胞构成,但是胰腺腺泡细胞癌只占胰腺外分泌肿瘤的1%～2%,最初由Berner于1908年报道[1]。该瘤确诊时一般体积已经很大,直径10～15 cm,且与胰腺内分泌肿瘤很难鉴别。本文报道1例发生于胰头钩突部位、体积较小的腺泡细胞癌,并结合文献讨论其病理特点及鉴别诊断。 Although 80% of the pancreas is composed of acinar cells, pancreatic acinar cell carcinoma accounts for only 1% to 2% of pancreatic exocrine tumors originally reported by Berner in 1908 [1]. The tumor is generally diagnosed when the volume has been very large, diameter 10 ~ 15 cm, and pancreatic endocrine tumors is difficult to identify. This article reports a case occurred in the uncinate process of the head of the pancreas, smaller acinar cell carcinoma, combined with the literature discuss its pathological features and differential diagnosis.