Reliable markers of PACC have been slowly emerging such as carcinoembryonic antigen (CEA) [15], cytokeratin 18 (CK18) [16] and B-cell lymphoma/leukemia 10 (BCL10) [17, 18] allowing us to distinguish acinar cell carcinoma from normal acinar cells or other pancreatic cancers through histology. The gene discussed is KRT18; the disease is familial pancreatic carcinoma.