Table 2.

Lifetime cancer risks and management recommendations for inherited colorectal cancer syndromes

Condition	Lifetime cancer risk		Screening recommendations	Initiation age
Lynch syndrome	Colorectum	60–80%	Colonoscopy: every 1–2 y	20–25 y
	Endometrium	40–60%	Transvaginal ultrasound ± endometrial	30 y
	Ovarian	9–12%	biopsy: annually; consider prophylactic TAH/BSO when childbearing complete
	Stomach	11–19%	EGD: every 2–3 years	30–35 y
	Upper urinary tract	4–5%	Urinalysis ± cytology: annually	30–35 y
	Pancreas	3–4%	There is currently no evidence to support screening for other Lynch syndrome related cancers
	Biliary tract	2–7%
	Small bowel	1–4%
	Central nervous system*	1–3%
Familial adenomatous polyposis (FAP)	Colorectum	100%	Flexible sigmoidoscopy; Annual colonoscopy when polyps detected	10–12 y
			Prophylactic colectomy when polyp burden not amenable to endoscopic resection
			Following colectomy: screen annually if remaining rectum or ileal pouch
	Duodenum/ampulla	4–12%	EGD: every 1–3 years	20–25 y
	Thyroid	1–2%	Annual thyroid palpation ± ultrasonography
	Hepatoblastoma	1–2%	Annual hepatic ultrasonography and AFP for the first five years of life	6 months
	Central nervous system**	<1%	There is currently no evidence to support screening for other related cancers
	Stomach	<1%
Attenuated familial adenomatous polyposis (AFAP)	Colorectum	70%	Colonoscopy: every 1–3 years	20–25 y
			Prophylactic colectomy when polyp burden not amenable to endoscopic resection
			Following colectomy: screen annually if remaining rectum or ileal pouch
	Duodenum/ampulla	4–12%	EGD every 1–3 years	20–25 y
	Thyroid	1–2%	Annual thyroid palpation ± ultrasonography
MYH-associated polyposis (MAP)	Colorectum	80%	Colonoscopy: every 1–2 years	25 y
			Prophylactic colectomy when polyp burden not amenable to endoscopic resection
			Following colectomy: screen annually if remaining rectum or ileal pouch
	Duodenum	4%	EGD every 1–3 years	30 y
Peutz-Jeghers syndrome (PJS)	Colorectum	39%	Colonoscopy: every 2–3 years	18 y
	Breast	55%	Mammography or breast MRI: annually	25 y
	Pancreas	36%	Endoscopic ultrasound or MRCP every 1–2 years	30 y
	Stomach	29%	EGD: every 2–3 years	8 y
	Lung	15%	No current recommendations
	Small bowel	13%	Small bowel imaging (capsule endoscopy, small bowel follow-through, CT or MRI enteroscopy): every 2–3 years	8 y
	Endometrium/cervix	9%	Annual pelvic examination, Papanicolaou smear, and transvaginal ultrasound	18 y
	Ovarian	21%
	Testicle	<1%	Annual testicular examination ± ultrasonography	10 y
Juvenile polyposis	Colorectum	40–50%	Colonoscopy: every 2–3 years	18 y
	Stomach	20%	EGD: annually when polyps are found otherwise every 2–3 years	18 y
	Small intestine	<1%	There is currently no evidence to support screening
Cowden syndrome	Breast	50%	Annual mammography or breast MRI	30–35 y
	Thyroid	10%	Annual thyroid ultrasonography	18 y
	Colorectum	16%	Colonoscopy every 3–5 years	35 y

TAH/BSO=total abdominal hysterectomy/bilateral salpingo-oophorectomy;

EGD=esophagoduodenoscopy; MRI=magnetic resonance imaging; MRCP=magnetic resonance cholangiopancreatography; CT=computed tomography.

^*Central nervous system tumors in Lynch syndrome include glioblastoma and astrocytomas

^**Central nervous system tumors in FAP include medulloblastoma