What is the best treatment for patients with Peutz-Jeghers syndrome?

Surgery. The gold standard is prophylaxis and polypectomy of the entire small bowel. Treatment of patients with inhibitors of cyclooxygenase-2 (COX-2 inhibitors, celecoxib) may lead to a dramatic reduction in the burden of polyps and need for surgical resection. Ongoing research indicates that Rapamycin (sirolimus) shows promise for PJS treatment by binding to FKBP12, which results in antiproliferative effects and dramatic reduction of polyp production/size.

Kopacova M, Tacheci I, Rejchrt S, Bures J: Peutz-Jeghers syndrome: diagnostic and therapeutic approach, World J Gastroenterol 15(43):5397–5408, 2009.