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Hereditary Cancer Syndromes >  Familial Polyposis APC

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CANCER

GENETIC CANCER RISK

Colorectal

High Risk

Gastric

Elevated Risk

Endocrine

Elevated Risk

Other

High Risk

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CANCER TYPE

AGE RANGE

CANCER RISK

RISK FOR GENERAL POPULATION

Colorectal

FAP to age 21

7%

<0.1%

FAP to age 50

93%

0.4%

FAP to age 80

>99%

 2.8%

AFAP to age 80

>70%

2.8%

Other - Desmoid Tumors

To age 80

10%-30%

<0.04%

Small Bowel/Periampullary

To age 80

4%-12%

0.2%

Hepatoblastoma

To age 5

1%-2%

<0.001%

Gastric

To age 80

1.3%, but significantly higher in Japanese and Korean populations

0.6%

Thyroid

To age 80

2.6%-8.5%

1.1%

Central Nervous System

To age 80

1%

0.5%

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

FREQUENCY

Colorectal

Colonoscopy

10 to 15 years

Annually

Colorectal surgical evaluation and counseling.

Based on cancer diagnosis and/or polyp number, size and histology

NA

Consider chemoprevention with NSAIDs to reduce adenoma burden after surgery.

NA

NA

Other - Desmoid Tumors

Abdominal MRI or CT

When symptoms are present

At least annually

Small Bowel/

Periampullary

Upper endoscopy, with consideration of capsule endoscopy to visualize the entire small bowel

20 to 25 years, or earlier if there is a family history of small bowel adenomas or cancer

Every 3 to 5 years

Hepatoblastoma

Consider liver palpation, abdominal ultrasound, and alpha-fetoprotein (AFP) measurement.

Infancy

Every 3 to 6 months during first 5 years of life

Gastric

Consider screening for patients with high risk features

Individualized

Individualized

Thyroid

Thyroid ultrasound

Late teens

Every 2 to 5 years, or consider more often if there is a family history of thyroid cancer

Central Nervous System

Patient education about the importance of quickly seeking attention for signs and symptoms of neurologic cancer

Individualized

NA

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