Surgical management of hereditary pancreatic cancer.

Pancreatic cancer continues to be a leading cause of cancer death in the United States. Seven genetic syndromes are now known to be associated with an increased incidence of pancreatic cancer. Other familial forms of pancreatic cancer exist although the genetic basis for this predisposition remains elusive. The similarities in the genetic and clinical manifestations of the sporadic and familial forms of pancreatic cancer suggest that pretreatment staging and management of patients with established pancreatic cancer should be similar. For carcinomas of the pancreatic head, pancreaticoduodenectomy should be performed according to current surgical practice, whereas the use of total pancreatectomy should be limited to cases in which margins are found to be positive or if the anatomy precludes a safe pancreaticojejunostomy. Total pancreatectomy may be considered in high-risk kindreds who strongly desire prophylactic surgery and in those with premalignant lesions. Identification of the precise genetic basis for inherited pancreatic cancer will someday make it possible to examine scientifically the effectiveness of specific management strategies.