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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, mucous 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. Text Continues Below
There are two types of PJS -- "familial" PJS, which is inherited, and "sporadic" PJS, which is not. Causes, incidence, and risk factors:
When inherited, Peutz-Jeghers syndrome is transmitted as an autosomal dominant trait. This means that, on average, each child of someone with familial PJS has a 50-50 chance of inheriting PJS.
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