After reporting a null finding from their randomized controlled trial of aspirin (acetylsalicylic acid – ASA) to prevent the<unk>  neoplasia of Lynch syndrome, Burn and colleagues have reported new data, representing a longer follow-up period than reported in the initial NEJM paper. These new data demonstrate a reduced incidence in people with Lynch syndrome who were exposed to at least four years of high-dose aspirin, with a satisfactory risk profile. These results have been widely covered in the media; future studies will look at modifying (lowering) the dose (to reduce risk associated with the high dosage of ASA). Screening Genetic counseling and genetic testing are recommended for families that meet the Amsterdam criteria, preferably before the onset of colon<unk> . Colon<unk>  Colonoscopies are recommended as a preventative method of surveillance for individuals who have Lynch syndrome, or LS-associated genes. Specifically, it is recommended that colonoscopies begin at ages 20–25 for MLH1 and MSH2 mutation carriers and 35 years for MSH6 and PMS2 mutation carriers. Colonoscopic surveillance should then be performed at a 1-2 year interval for Lynch Syndrome patients. Endometrial/Ovarian<unk>  A transvaginal ultrasound with or without endometrial biopsy is recommended annually for ovarian and endometrial<unk>  screening. For women with Lynch syndrome, a yearly CA-125 blood test can be used to screen for ovarian<unk> , however there is limited data on the efficacy of this test in reducing mortality. Other<unk> s There are also strategies for detecting other<unk> s early or reducing the chances of developing them that people with Lynch syndrome can discuss with their doctor, however their effectiveness is not clear. These options include: * Upper endoscopies to detect stomach and small bowel<unk>  every 3–5 years, starting at age 30 at the earliest (preferably in a research setting) * Annual urinalysis to detect bladder<unk> , starting at age 30 at the earliest (preferably in a research setting) * Annual physical and neurological exams to detect<unk>  in the central nervous system (brain or spinal cord), starting at age 25 at the earliest Amsterdam criteria The following are the Amsterdam criteria in identifying high-risk candidates for molecular genetic testing: Amsterdam I Criteria (all bullet points must be fulfilled): The Amsterdam I criteria were published in 1990; however, were felt to be insufficiently sensitive. * Three or more family members with a confirmed diagnosis of<unk><unk> , one of whom is a first degree (parent, child, sibling) relative of the other two * Two successive affected generations * One or more colon<unk> s diagnosed under age 50 years * Familial adenomatous polyposis (FAP) has been excluded The Amsterdam II criteria were developed in 1999 and improved the diagnostic sensitivity for Lynch syndrome by including<unk> s of the endometrium, small bowel, ureter and renal pelvis. Amsterdam Criteria II (all bullet points must be fulfilled): * Three or more family members with<unk> -related<unk> s, one of whom is a first-degree relative of the other two * Two successive affected generations * One or more of the<unk> -related<unk> s diagnosed under age 50 years * Familial adenomatous polyposis (FAP) has been excluded The Bethesda criteria were developed in 1997 and later updated in 2004 by the National<unk>  Institute to identify persons requiring further testing for Lynch syndrome through MSI. In contrast to the Amsterdam Criteria, the Revised Bethesda Guidelines use pathological data in addition to clinical information to help health care providers identify persons at high risk. Revised Bethesda Guidelines If a person meets any 1 of 5 criteria the tumour(s) from the person should be tested for MSI: 1.<unk><unk>  diagnosed before age 50 2. Presence of synchronous or metachronous<unk>  or other Lynch syndrome associated<unk> s (e.g.<unk> s of endometrium, ovary, stomach, small bowel, pancreas, biliary tract, ureter, renal pelvis, brain, sebaceous glands, keratoacanthomas) 3.<unk><unk>  with MSI-high pathology in a person who is younger than 60 years of age 4.<unk><unk>  diagnosed in a person with one or more first-degree relative with<unk><unk>  or Lynch syndrome associated tumour diagnosed under age 50 5. Person with<unk><unk>  and two or more first- or second-degree relatives with<unk><unk>  or Lynch syndrome associated<unk>  diagnosed at any age. It is important to note that these clinical criteria can be difficult to use in practice and clinical criteria used alone misses between 12 and 68 percent of Lynch syndrome cases. Surgery Prophylactic hysterectomy and salpingo-oophorectomy (removal of the uterus, Fallopian tubes, and ovaries to prevent<unk>  from developing) can be performed before ovarian or endometrial<unk>  develops.
Hereditary nonpolyposis colorectal cancer
Familial adenomatous polyposis
MUTYH-associated polyposis
Serrated polyposis syndrome
Hereditary diffuse gastric cancer
Juvenile polyposis syndrome
Attenuated familial adenomatous polyposis
Peutz–Jeghers syndrome