Pathogenesis of adenocarcinoma in Peutz-Jeghers syndrome.

  title={Pathogenesis of adenocarcinoma in Peutz-Jeghers syndrome.},
  author={Stephen B. Gruber and Mark M. Entius and G. M. van Petersen and Steven J. Laken and Patti A. Longo and Rebecca Boyer and Albert M. Levin and Urvi J. Mujumdar and Jeffrey M. Trent and Kenneth W. Kinzler and Bert Vogelstein and Stanley R. Hamilton and Mihael H. Polymeropoulos and G. Johan A. Offerhaus and Francis M. Giardiello},
  journal={Cancer research},
  volume={58 23},
Peutz-Jeghers syndrome (PJS) is an autosomal dominant condition characterized by intestinal hamartomatous polyps, mucocutaneous melanin deposition, and increased risk of cancer. Families with PJS from the Johns Hopkins Polyposis Registry were studied to identify the molecular basis of this syndrome and to characterize the pathogenesis of gastrointestinal hamartomas and adenocarcinomas in PJS patients. Linkage analysis in the family originally described by Jeghers in 1949 and five other families… CONTINUE READING
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