How is Peutz Jeghers syndrome treated?

General: There is no cure for Peutz Jeghers syndrome (PJS). Instead, treatment aims to reduce symptoms and prevent complications. Depending on individual symptoms and complications, individuals with PJS should be regularly seen by a geneticist, gynecologist, oncologist, dermatologist, and gastroenterologist.

Cancer treatment: People with PJS who develop cancer should receive standard cancer treatment. This often includes radiation and chemotherapy. By damaging the deoxyribonucleic acid (DNA) in the target cells, radiation therapy stops the growth of cancerous cells. While both normal and cancer cells are damaged by radiation, normal cells can typically recover, while cancer cells cannot. The goal of radiation therapy is to maximize damage to cancer cells and minimize damage to normal cells. About half of those with cancer receive radiation therapy in some form. Side effects of radiation therapy depend on the site of the radiation target and the type of cancer. For example, nausea, vomiting, and diarrhea may result from radiation therapy targeted at the stomach and related areas. Radiation may be used alone or in combination with other therapies, such as chemotherapy, which involves the use of drugs to stop cancerous cell growth. Again, however, chemotherapy can damage normal cells as well as cancerous cells. Side effects of chemotherapy may include nausea, vomiting, fatigue, pain, and loss of hair on the head and body.

Iron supplementation: Iron supplementation may be prescribed to individuals who develop iron deficiency anemia, a blood disorder characterized by low levels of red blood cells.

Surgery: Surgical removal of large polyps may be appropriate to reduce pain, bleeding, and risk of bowel obstruction. Surgery is also an option for the removal of cancerous growths.

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