LYNCH SYNDROME

Course overview

MOST COLORECTAL CANCERS OCCUR SPORADICALLY; HOWEVER, INHERITED CANCER SYNDROMES OR INHERITED MUTATIONS CAUSE APPROXIMATELY 5 TO 10 PERCENT OF CASES. THE MOST COMMON HEREDITARY FORM OF HEREDITARY COLORECTAL CANCER IS LYNCH SYNDROME, ALSO KNOWN AS HEREDITARY NON-POLYPOSIS COLORECTAL CANCER SYNDROME (HNPCC). IDENTIFYING PATIENTS WITH LYNCH SYNDROME IS CLINICALLY IMPORTANT BECAUSE THESE PATIENTS HAVE UP TO 80 PERCENT LIFETIME RISK OF COLORECTAL CANCER AND UP TO 60 PERCENT LIFETIME RISK OF ENDOMETRIAL CANCER. THESE PATIENTS ALSO HAVE AN INCREASED RISK FOR OTHER PRIMARY CANCERS INCLUDING GASTRIC, OVARIAN, SMALL BOWEL, UROTHELIAL (URETER, RENAL PELVIS), BILIARY TRACT, PANCREATIC, BRAIN (GLIOBLASTOMA), SEBACEOUS GLAND ADENOMAS, AND KERATOACANTHOMAS. THIS ACTIVITY DESCRIBES THE EVALUATION, DIAGNOSIS, AND MANAGEMENT OF LYNCH SYNDROME AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN THE CARE OF AFFECTED PATIENTS.