Acinar cell cystadenocarcinoma of the pancreas in a 4-year-old child.

To the Editor: Cystic neoplasms of the pancreas are rare, constituting only 10% of all cystic lesions of the pancreas in the adult population. These neoplastic pancreatic cysts can be divided into serous adenomas, mucinous cystadenomas, and cystadenocarcinomas. Acinar cell cystadenocarcinomas (ACCCs) are a very rare variant of acinar cell adenocarcinoma of the pancreas, of which only 7 cases have been reported. All these cases occurred in adults. No child was reported to have this disease. We now describe the clinicopathologic features of a 4-year old child with ACCC and review the relevant literature.