Acinar cell cystadenocarcinoma of the pancreas: Report of rare case and review of the literature

Most exocrine pancreatic tumors are of ductal origin, whereas acinar cell adenocarcinomas are unusual (1% to 2% of all exocrine pancreatic neoplasms). We recently found a cystic adenocarcinoma of the pancreatic body whose cells had the characteristics of acinar cells, which we term acinar cell cystadenocarcinoma. Macroscopically, this tumor consists of a large multilocular cystic mass with a pseudocapsule and a spongy appearance on the cut surface. Microscopically, the cysts are lined by a single layer of cuboid/columnar cells. The cytoplasm has the characteristics of acinar cells, with eosinophilic granules in the apex and prominent nucleoli. Immunohistochemically, the cells express α1-antitrypsin, trypsin, and lipase in their cytoplasm, thus confirming the acinar origin of the tumor. A review of the literature revealed only 5 other cases of this tumor reported since its first description in 1981. Follow-up data are available for 4 of these; all of the affected patients had metastases at presentation or a few months later, and 2 died of the disease, at 13 and 37 months after diagnosis. Although this variant of adenocarcinoma of the pancreas is not prognostically different from the classic solid type (few patients survive more than 5 years), we believe that it is important because of its extreme rarity.