Three subjects were surprisingly found to carry genetic alterations associated with well-established genetic syndromes not usually related to desmoid tumors: two Lynch syndrome patients carried MSH6 mutation and VUS in MSH2 and one patient had a mutation in PALB2, which encodes a BRCA2 interacting protein that increases susceptibility to breast and pancreatic cancers [40]. This evidence concerns the gene MSH6 and familial pancreatic carcinoma.