Copyright intraductal papillary mucinous neoplasia. Even early-stage pancreatic cancer still maintains a poor prognosis; therefore, early detection strategies should be aimed at detecting premalignant lesions rather than cancer itself. Several… Click to show full abstract
Copyright intraductal papillary mucinous neoplasia. Even early-stage pancreatic cancer still maintains a poor prognosis; therefore, early detection strategies should be aimed at detecting premalignant lesions rather than cancer itself. Several institutions have developed programs for high-risk populations, including the University of Texas MDAnderson Cancer Center (MDACC), in which patients with germline mutations at increased susceptibility for PDAC or with a strong family history of PDAC undergo regular surveillance. Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder with a mutation in the serine-threonine kinase 11/liver kinase B1 (STK11/LKB1) tumor suppressor gene located on chromosome 19p13.3. Peutz-Jeghers syndrome is associated with a 132-fold increase risk for PDAC as compared with the general population and is clinically characterized by mucocutaneous pigmentation, gastrointestinal hamartomatous polyps, and predisposition to lung, breast, gastrointestinal, and gynecological malignancies.
               
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