Chapter 15 Brain Tumors and the Lynch Syndrome

Lynch syndrome (LS) (MIM No. 120435-6), previously known as hereditary nonpolyposis colorectal cancer (HNPCC) (Boland, 2005), is an autosomal dominant disorder caused by

germline mutation in one of the DNA mismatch repair (MMR) genes. LS is among the most

prevalent cancer syndromes in man and is estimated to account for 1-6% of all colorectal

cancers (Lynch & de la Chapelle, 2003).