Lynch syndrom type 2

Hi, From the precise nature of your question, I can sense that you are well-informed about hereditary cancers. For the benefit of other visitors of this website, I would like to elaborate a bit on the epidemiology and genetics of inherited colo-rectal cancers (CRC). According to Fearon and Bommer in the 8th edition of deVita's Principle and Practice of Oncology, most CRC's occur sporadically (caused by a random mutation). About 15 to 30% have may have a hereditary component. Those with a first-degree relative with CRC has twice the chance of developing this cancer as compared to those who do not have a positive family history. Additionally, some specific cancer syndromes have been described, which may account for about 5% of all CRC. These syndromes include Familial Adenomatous Polyposis (FAP, accounting for about 0.5% of all CRC), hereditary nonpolyposis CRC (HNPCC, accounting for about 2-4% of all CRC), Peuts Jeghers syndrome, Cowdens Syndrome, and Juvenile Polyposis syndrome. HNPCC is also called Lynch Syndrome. Apart from CRC, families with Lynch syndrome may develop endometrial, ovarian, gastric, heaptobiliary, and urinary tract cancers. Several criteria (like Amsterdam criteria, Bathesda criteria) are used to define HNPCC and to identify patients who need further genetic testing. Lifetime risk for CRC in HNPCC is 80%, about 40% for endometrial cancers, and about 10% for other cancers (Reference: Libutti, Saltz, Tepper, in deVita 8th Edition). For colon cancer screening, colonoscopies are recommended starting at age 20-25 or 10 years prior to youngest age of diagnosed family member, and should be repeated every 1 to 2 years. For endometrial cancer, seek close surveillance under the care of a gynecological oncologist. Prophylactic surgery (removal of uterus, fallopian tubes, and ovaries) is to be discussed with women who have completed childbearing. For screening for gastric cancer, upper GI endoscopy is recommended, starting age 25-30, to be repeated every 1 to 3 years. For screening for urothelial cancers, annual urine tests are recommended. (Reference: NCCN Guidelines). For screening for other cancers, local institutional policies may be followed. All the very best, and God Bless!

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