The major genetic alterations leading to sporadic pancreatic cancer are thought to be mutations in the proto-oncogene, KRAS, as well as the p16/CDKN2A/INK4A, TP53, and DPC4/SMAD4 tumor suppressor genes, while mutations in BRCA2, the mismatch repair genes (hMLH1, hMSH2, and hMSH6), and the AKT2 and STK11/LKB1 genes are noted to be rare (Schneider and Schmid, 2003; Hezel et al., 2006). The gene discussed is STK11; the disease is familial pancreatic carcinoma.