What is Peutz-Jeghers syndrome (PJS)?
PJS is another rare polyposis syndrome. This condition often runs in families but may occur without any known family member affected. PJS often causes skin pigmentation (i.e., freckles) that are most commonly seen around the mouth, lips, and on the inside of the cheeks. Having many freckles in these areas is unusual in kids without this disorder. PJS polyps may occur anywhere in the gastrointestinal tract. These polyps are often large and may cause intussuception with intestinal blockage. These children often require multiple surgeries due to intussuception. These children often need multiple endoscopy procedures to remove polyps. It is unclear to what degree PJS polyps can become cancerous. Kids with PJS are at higher risk than the general population for development of cancers both in the gastrointestinal tract and in other body areas. These children should be monitored closely for all types of cancers (i.e., testicular cancer in boys).
