II. Types
- Familial Adenomatous Polyposis (Adenomatous Polyposis Coli, MYH-Associated polyposis)
- Synchronous advanced adenomas numbering >100
- Prevalence: 3 in 100,000
- Diagnosed on average by age 39 years old
- Colonrectal cancer develops in 87% by age 45 years old (without colectomy)
- Attenuated Familial Adenomatous Polyposis
- Synchronous advanced adenomas numbering 10-99
- Diagnosed on average by age 58 years
- Colorectal Cancer develops 69% by age 80 years old (without colectomy)
- MUTYH-Associated Polyposis (<100 Colorectal Adenomas)
- Autosomal Recessive risk for Colorectal Cancer
- Prevalence: <1 in 10,000 (U.S.)
- Colorectal Cancer presents typically by age 50-70 years old
- Colorectal Cancer risk 19% by age 50, 43% by age 60 years and 100% lifetime risk
III. Management
- Familial Adenomatous Polyposis (>100 synchronous advanced adenomas)
- Counseling to consider Genetic Testing
- Early surveillance
- Flexible Sigmoidoscopy starting at Puberty (age 10-12 years)
- Repeat Flexible Sigmoidoscopy every 1-2 years until polyp found, then Colonoscopy
- Colonoscopy annually until colectomy
- Genetic Testing positive or polyposis confirmed
- Consider colectomy or
- Endoscopy every year
- Genetric testing negative (or not performed) and no polyposis
- Screen every year until age 24 years, then
- Screen every 2 years from age 24 to 34 years old, then
- Screen every 3 years until age 44 years, then
- Screen every 3-5 years after age 44 years old
- Attenuated Familial Adenomatous Polyposis (10-99 synchronous advanced adenomas)
- Colonoscopy every 1-2 years starting by late teens to early 20s
- Proximal polyp distribution (Flexible Sigmoidoscopy is insufficient)
- Colonoscopy every 1-2 years starting by late teens to early 20s
- MUTYH-Associated Polyposis (<100 Colorectal Adenomas)
- Colonoscopy starting by late teens to early 20s and repeated every 1-2 years
- Colectomy when polyps cannot be controlled with endoscopy
IV. Management: Other screening
- Upper endoscopy (for Gastric Cancer, proximal Small Bowel tumor) starting at age 25 years
- Annual Thyroid exam and consider thyroid Ultrasound