Timing and extent of surgical intervention in patients from hereditary pancreatic cancer kindreds.

Our knowledge of the molecular and genetic etiology of hereditary pancreatic cancer has expanded considerably and is steadily increasing. However, there are only a few hard data available regarding the clinical and surgical management of these patients. Surgery is currently performed when we detect dysplastic changes in the pancreas or when cancer is suspected. Of the available diagnostic modalities, endoscopic ultrasonography has proven so far to be the most useful for detecting dysplastic changes in the pancreases of patients from hereditary pancreatic cancer kindreds. It seems reasonable, once dysplasia has been diagnosed in a high-risk patient, to proceed to total pancreatectomy. The multifocal nature of dysplastic lesions precludes any type of operation that would leave behind pancreatic tissue. Currently, prophylactic whole-organ resection in the absence of premalignant lesions cannot be recommended since we do not know the exact risk for the development of cancer.