Only 10% of PDACs are linked to genetic mutations such as BRCA2, STK11/LKB1, CFTR, and PRSS1 or to familial syndromes such as Von Hippen-Lindau disease (VHL), multiple endocrine neoplasia syndrome type 1 (MEN-1), and neurofibromatosis type 1 (NF-1). Here, STK11 is linked to von Hippel-Lindau disease.