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Hereditary Cancer Syndromes >  Familial Polyposis APC

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CANCER

GENETIC CANCER RISK

Colorectal

High Risk

Gastric

Elevated Risk

Pancreatic

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

Slightly increased risk in Western cultures, but may be significantly increased in cultures with a higher baseline gastric cancer rate.

0.6%

Thyroid

To age 80

2.6%-8.5%

1.2%

Central Nervous System

To age 80

1%

0.5%

Pancreatic

To age 80

Possibly elevated risk

1.1%

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

FREQUENCY

Colorectal

Sigmoidoscopy or 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

Pancreatic

Consider screening for patients with a family history of pancreatic cancer

Individualized

Individualized

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