___________________________________________________________________________ Colorectal Cancer
SCREENING AND SURVEILLANCE RECOMMENDATIONS FOR COLOREC TAL CANCER AND EXTRACOLONIC MALIGNANCIES IN PATIENTS WITH HEREDITARY COLORECTAL CANCER SYNDROMES Cancer Screening Recommendations Lynch syndrome/HNPCC Colorectal Colonoscopy every one to two years starting at 20 to 25 years of age or two to five years before earliest colorectal cancer in the family if diagnosed before 25 years of age Gastric and small bowel Consider baseline EGD beginning at 40 years of age and surveillance EGD every three to five years Urothelial Annual urinalysis may begin at 30 to 35 years of age CNS Annual physical exam, no added screening Pancreatic Consider screening beginning at 50 years of age (or 10 years younger than earliest exocrine pancreatic cancer diagnosis in the family, whichever is earlier) for individuals with exocrine pancreatic cancer in ≥1 first- or second-degree relatives from the same side of the family as the identified pathogenic/likely germline variant
Endometrial and ovarian (women)
Endometrial sampling every one to two years beginning at 30 to 35 years of age Transvaginal ultrasound is not recommended May consider prophylactic hysterectomy and bilateral salpingo-oophorectomy after childbearing is completed
Diagnosis of familial adenomatous polyposis (FAP) Colorectal: APC gene-positive Flexible sigmoidoscopy or colonoscopy annually starting at 10 to 15 years of age, then every two to three years Consider colectomy Colorectal: Suspected FAP, not tested Flexible sigmoidoscopy or colonoscopy starting 10 to 15 years of age, then annually until 24 years of age, every two years until 34 years of age, and every three years until 44 years of age, then every three to five years thereafter
Personal history of FAP, post-colectomy Colorectal
Endoscopic evaluation every six months to three years, depending on proctocolectomy or colectomy status NSAID chemoprevention to reduce polyp burden as pharmacologic adjunct to endoscopy Baseline upper endoscopy (including side-viewing exam), beginning at 20 to 25 years of age, repeated every one to three years depending on severity of polyposis Examine stomach at time of duodenoscopy
Duodenal, gastric, or periampular
Thyroid
Annual thyroid exam starting in late teens Annual physical exam, no added screening
CNS cancer
Intra-abdominal desmoids
Annual abdominal palpation With a family history of desmoids, consider abdominal CT or MRI every 1 to 3 years post- colectomy and then at 5- and 10-year intervals Add small bowel visualization with CT or MRI for desmoids as outlined above, especially with advanced duodenal polyps Liver palpation, abdominal ultrasound, and measurement of α -fetoprotein every three to six months until 5 years of age FAP genetic testing in untested children with hepatoblastoma
Small bowel polyps and cancer Hepatoblastoma (childhood cancer associated with FAP)
Pancreatic
No recommendations
Personal history of AFAP Colorectal: <21 years, small adenoma burden
Colonoscopy and polypectomy every one to two years; surgical evaluation and counseling
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MDFL2626
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