Surgical management of hereditary pancreatic cancer

Unlike other inherited cancers, patients with a genetic predisposition to pancreatic cancer have a similar age of onset and prognosis as those with the sporadic form of the disease. Given these similarities, recommendations regarding evaluation and management are similar for both groups. Accurate pretreatment staging is critical to minimizing treatment-associated morbidity and achieving the best possible results. Surgery remains the only chance for a cure of pancreatic cancer and has evolved considerably during this century. The standard Whipple procedure with postoperative adjuvant chemoradiation 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 preventive management strategies.