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Peutz-Jeghers-Syndrom

Nicht immer nur eine Polyposis!

Peutz-Jeghers syndrome

Not only a polyposis!

Zusammenfassung

Die klinischen Zeichen eines Peutz-Jeghers-Syndroms gehen nicht selten in der Allgemeinversorgung unter. Die frühzeitige Diagnosestellung ist jedoch für die weitere Prognose maßgeblich. Hier ist es v. a. wichtig, nicht nur an die bekannte Manifestation der gastrointestinalen Polyposis zu denken, sondern auch an andere, die Prognose mitunter stark beeinflussende Prozesse wie die hier beschriebenen assoziierten Tumoren (z. B. Ovarial-, Hodentumoren) bei Vorliegen einer Mutation des Serin-Threonin-Kinase-Gens STK11 (Chromosom 19p13.3).

Abstract

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.

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Correspondence to S. Greven.

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Greven, S., Fölster-Holst, R. Peutz-Jeghers-Syndrom. Hautarzt 63, 877–879 (2012). https://doi.org/10.1007/s00105-012-2411-2

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  • DOI: https://doi.org/10.1007/s00105-012-2411-2

Schlüsselwörter

  • Polyposis
  • Peutz-Jeghers-Syndrom
  • Tumoren
  • Pigmentflecken
  • Sertoli-Zell-Hyperplasie

Keywords

  • Polyposis
  • Peutz-Jeghers-syndrome
  • Tumors
  • Lentigines
  • Sertoli cell hyperplasia