Table 7 -- Risk and Surveillance of Cancers associated with Hamartomatous Polyposis
Syndrome
Cancer
Genetic Test
Intervention
Surveillance Recommendations
Juvenile Polyposis #
Stomach

Or

Small bowel

Or

Colon
no ##
EGD

Or

UGI with SBFT

Or

Colonoscopy
**Patients with polyps should have surveillance intervals of Q1-2 yrs
Cowden
Breast

Thyroid

Skin
no*
Mammograms
TFT/thyroid scan
Physical exam
Every 6-12 months beginning in mid-20s
Peutz-Jegher
Entire GI tract especially Small Bowel
no~
EGD
UGI with SBFT
Colonoscopy
Every 2 years post-diagnosis; remove polyps esp. if >1.5 cm

# There are three forms of juvenile polyposis (JP) based on the location of the polyps.

## The PTEN or DPC4 genes can be mutated in Juvenile Polyposis; testing is not yet available however.

* A germ-line mutation PTEN has been described in families with Cowden disease but commercial testing is not available yet.

** Surveillance should be tailored to the type of GI cancer the family inherits (stomach vs. colon).


~ The LKB1/STK11 gene on Chromosome 19p has been associated with PJ but commercial testing is not yet available.