The risk for breast and ovarian cancer is increased with Peutz-
Jeghers syndrome (PJS), a rare early-onset autosomal dominant
disorder, associated with specific physical characteristics in
addition to increased cancer risks. The features associated with
PJS may include the following: Peutz-Jeghers syndrome is caused
by mutations in a gene on chromosome 19 known as STK11. Genetic
testing is available clinically. Mutations in STK11 are
identified in nearly all individuals with a positive family
history and about 90 percent of individuals with no previous
family history. Almost all people with PJS will be diagnosed
with one or more of the associated cancers during their
lifetime. The STK11 gene is a tumor suppressor gene, which
usually controls cell growth and cell death. Both copies of a
tumor suppressor gene must be altered, or mutated, before a
person will develop cancer. With PJS, the first mutation is
inherited from either the mother or the father and is present
from birth in all cells of the body. This is c