Acinar cystic transformation in the pancreatic tail

Pancreatic acinar cystic transformation (ACT) is a rare non-neoplastic cystic lesion that is predominantly located at the pancreatic head in females. Preoperative definitive diagnosis of ACT remains challenging despite advances in radiologic imaging methods. A 25-year-old male patient presented with abdominal discomfort and a 50-mm cystic lesion in the pancreatic tail. The patient underwent laparoscopic distal pancreatectomy, because branch duct intraductal papillary mucinous neoplasm cannot be ruled out and the presence of abdominal symptoms. The resected specimen revealed a collection of small and large cysts lined by a single cuboidal epithelium layer with scattered pancreatic tissue exhibiting fibrosis in the septal wall. The cystic lesion was epithelial, trypsin-positive, B cell lymphoma 10-positive, cytokeratin 19-positive, mucin 1-positive, and MUC6-negative with a differentiated lobular central conduit causing to an adeno-cystic cell, thereby supporting the ACT diagnosis. Distinguishing ACT from other pancreatic cystic tumors remains a diagnostic challenge despite improvements in radiologic imaging methods. Surgical resection may be justified when other cystic neoplasms cannot be excluded because of its heterogeneous nature, although the ACT is a non-neoplastic lesion, and cases of malignant transformation have never been reported to date.