Hereditary Cancer Syndromes >  Familial Polyposis APC

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CANCER

GENETIC CANCER RISK

Colorectal

High Risk

Gastric

Elevated Risk

Pancreatic

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%

0.3%

FAP to age 80

>99%

3.0%

AFAP to age 80

>70%

3.0%

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

1%-2%

1.1%

Central Nervous System

To age 80

1%

0.5%

Pancreatic

To age 80

Possibly elevated risk

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 palpation with consideration of abdominal MRI or CT.

1 to 3 years post-colectomy

Every 5 to 10 years, or with symptoms

Small Bowel/Periampullary

Upper endoscopy, including complete visualization of the ampulla of Vater

20 to 25 years, or earlier if patient had a colectomy before age 20 years

Every 4 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

Upper endoscopy

20 to 25 years, or earlier if patient had a colectomy before age 20 years

Every 4 years

Thyroid

Thyroid examination and/or consider ultrasound.

Late teens

Annually

Central Nervous System

Physical examination

Individualized

Annually

Pancreatic

Currently there are no specific medical management guidelines for pancreatic cancer risk in mutation carriers.

NA

NA

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