| 34 35 | See page 67 for references Colorectal cancer surveillance and surgical options for affected individuals: · ·Colonoscopy and polypectomy every 1-2 years. · · Colectomy should be performed once polyp burden can no longer be managed by polypectomy alone. In most cases, colectomy with ileorectal anastomosis is preferred, but total proctocolectomy with ileal pouch-anal anastomosis may be considered if rectal polyp burden cannot be managed. · · In patients with colectomy with ileorectal anastomosis, endoscopic evaluation of the rectum every 6-12 months, depending on polyp burden is recommended. Extracolonic surveillance: · ·Conduct annual physical. · ·Consider upper endoscopy (including complete visualization of ampulla of Vater) beginning at age 30–35 years. There are currently no guidelines specific to the other potential cancer risks associated with MAP. Cancer risks For heterozygous carriers: There may be a moderately increased risk of developing colorectal cancer. The risk was found to be marginally increased in large population studies (OR 1.1-1.2), whereas family-based studies found a higher risk to heterozygote family members than in the general population (OR 2-3). For biallelic MUTYH mutations: The following table breaks down the different associated cancers. Cancer Lifetime risk* Colon 80%–100% Intestinal (small bowel) 4% Ovarian, bladder, breast, and endometrium Unknown Information in table from: Nielsen et al (2012, updated 2015). *These risks do not apply to carriers of a single MUTYH mutation. Potential management options NCCN recommends that physicians or centers with expertise in hereditary polyposis syndromes manage patients with MAP. Management should be individualized based on clinical manifestations and other personal considerations. Carriers for a single MUTYH mutation: · ·For individuals with a first-degree relative with colon cancer: colonoscopy screening every 5 years, beginning at age 40 or 10 years earlier than the age at which colon cancer was diagnosed in a first-degree relative. · ·For individuals with no family history of colon cancer: data are uncertain if specialized screening is warranted. Individuals with two (biallelic) MUTYH mutations: Colorectal cancer surveillance and surgical options for unaffected individuals: · ·Perform colonoscopy beginning at age 25–30 years. · ·If negative, repeat colonoscopy every 2–3 years. · ·If polyps are found, repeat colonoscopy every 1–2 years and manage with polypectomy. · ·Consider surgical evaluation based on polyp burden and age (see “Colorectal cancer surveillance and surgical options for affected individuals”).