Lynch Syndrome (Hereditary Nonpolyposis Colorectal Cancer, HNPCC)


Epidemiology/Genetics


Physiology

Normal DNA Mismatch Repair (MMR) Process

Lynch Syndrome Results from a Germline Mutation in One Allele of an MMR (or Related) Gene with the Second Allele Inactivated by Mutation, Loss of Heterozygosity, or Epigenetic Silencing by Promoter Methylation

Biallelic Inactivation of an MMR (or Related) Gene Causes a Failure of the DNA Repair Process, Resulting in an Increased Mutation Rate (Genomic Instability) and Increased Cancer-Susceptibility


Diagnosis


Amsterdam II Criteria for Lynch Syndrome [MEDLINE]


Clinical Manifestations

Gastrointestinal Manifestations

Colorectal Cancer (see Colorectal Cancer, [[Colorectal Cancer]])

Small Intestinal Cancer

Renal Manifestations

Reproductive Manifestations

Endometrial Cancer (see Uterine Cancer, [[Uterine Cancer]])

Other Manifestations


Treatment

Aspirin Prophylaxis (see Acetylsalicylic Acid, [[Acetylsalicylic Acid]])


References