Unlike other inherited cancers, patients with a genetic predisposition to p
ancreatic cancer have a similar age of onset and prognosis as those with th
e sporadic form of the disease. Given these similarities, recommendations r
egarding evaluation and management are similar for both groups. Accurate pr
etreatment staging is critical to minimizing treatment-associated morbidity
and achieving the best possible results. Surgery remains the only chance f
or a cure of pancreatic cancer and has evolved considerably during this cen
tury. The standard Whipple procedure with postoperative adjuvant chemoradia
tion is the mainstay of modern treatment. Routine total pancreatectomy for
patients with established pancreatic cancer cannot be recommended, although
continued research into familial cancer families may define its role in pr
eventive management strategies.