PEUTZ-JEGHERS SYNDROME

Course overview

PEUTZ-JEGHERS SYNDROME (PJS) IS A HEREDITARY SYNDROME CHARACTERIZED BY GASTROINTESTINAL (GI) POLYPOSIS, MUCOCUTANEOUS PIGMENTED MACULES, AND PREDISPOSITION TO CERTAIN CANCERS. PATIENTS WITH PEUTZ-JEGHERS SYNDROME ARE AT AN INCREASED RISK FOR DEVELOPING GI CANCERS OF THE COLORECTAL, PANCREATIC, AND GASTRIC SYSTEMS, IN ADDITION TO A WIDE VARIETY OF NON-GI EPITHELIAL MALIGNANCIES INCLUDING BREAST, UTERINE, CERVICAL, LUNG, OVARIAN, AND TESTICULAR CANCERS. AMONG THE VARIOUS MALIGNANCIES, COLORECTAL IS MOST COMMON WITH A LIFETIME RISK OF 39 PERCENT, AND IS FOLLOWED BY BREAST CANCER IN FEMALES WITH A LIFETIME RISK OF 32 TO 54 PERCENT. THIS ACTIVITY REVIEWS THE EVALUATION, MANAGEMENT AND SURVEILLANCE FOR PEUTZ-JEGHERS SYNDROME AND HIGHLIGHTS THE ROLE OF AN INTERPROFESSIONAL TEAM IN IMPROVING CARE FOR PATIENTS WITH THIS CONDITION.