[Peutz-Jeghers syndrome : not only a polyposis!].

The clinical signs of the Peutz-Jeghers syndrome are often overlooked in daily clinical practice. Early diagnosis is enormously relevant for the patient's outcome. Therefore, it is very important not only to think of the well-known manifestation of the gastrointestinal polyposis but also to consider the various other processes which can influence the prognosis remarkably as for instance ovarian and testicular tumors when a mutation of the serine-threonine kinase gene STK11 (chromosome 19p13.3) is present.