Hereditary Cancer Syndromes > Familial Polyposis APC

Cancer Risks

Cancer Risks

Cancer Risks

Associated Syndrome

Associated Syndrome

APC gene Cancer Risk Table

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 51 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%
Central Nervous System To age 80 1% 0.5%
Pancreatic To age 80 Possibly elevated risk 1%

APC Cancer Risk Management Table

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

Medical Management

Medical Management