Peutz-Jeghers syndrome natural history, complications, and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain. Common complications of Peutz-Jeghers syndrome include colon cancer, and cachexia.[1] Prognosis is generally good if polypectomy was performed before any dysplastic degenerations take place.

Natural History

Without polypectomy, patients with Peutz-Jeghers syndrome may develop symptoms of colon cancer, which will eventually lead to death.

Complications

Complications that can develop as a result of Peutz-Jeghers syndrome are:

Prognosis

Almost half of Peutz-Jeghers patients die from cancer by age 57 years, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.[2]

References

  1. Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.
  2. "eMedicine - Peutz-Jeghers Syndrome : Article by Andrea Duchini, MD". Retrieved 2007-07-21.

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