Peutz-Jeghers Syndrome
Definition
Peutz-Jeghers syndrome (PJS) is a disorder characterized by intestinal polyps. In addition, pigmented spots develop from infancy through childhood around the lips, gums, mucus membranes, and skin.
The polyps develop most commonly in the small intestine, but also in the colon. There may be a significant risk of these polyps becoming malignant and thus of patients developing cancer. Some studies link PJS and cancers of the gastrointestinal tract, lung, breast, uterus, and ovaries.
There are two types of PJS -- "familial" PJS is inherited, and "sporadic" PJS means there is no known hereditary link.
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Overview, Causes, & Risk Factors
When inherited, Peutz-Jeghers syndrome is transmitted as an autosomal dominant trait. This means that on average, half of the children of someone with familial PJS will inherit PJS.
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