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Extraintestinal polyps in Peutz-Jeghers syndrome: presentation of four cases and review of the literature

  • T. Vogel
  • V. Schumacher
  • A. Saleh
  • J. Trojan
  • G. Möslein
  • for the Deutsche
  • Peutz-Jeghers-Studiengruppe
Case Report

Abstract 

Peutz-Jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. Extraintestinal polyps have rarely been reported. Possible sites include the respiratory tract, urogenital tract, and gallbladder. We here describe four cases of extraintestinal polyps in PJS patients and review the literature on the need for operative therapy of extraintestinal polyps in PJS. Three nonrelated patients were examined who had PJS and polyps in the gallbladder; the fourth patient had PJS and recurrent choanal polyps. Surgery has so far been performed only for symptomatic polyps: one laparoscopic cholecystectomy and removal of the choanal polyps for recurrent infections of the respiratory tract. The remaining two patients reported no symptoms from the extraintestinal polyps. No malignant transformation was found in these patients, nor has such been reported in the literature on PJS. The frequent observation of this manifestation in our patients raises the question of clinical management: Is prophylactic surgery indicated? Since malignant transformation of PJS polyps in the intestine is extremely rare we see no reason for operative therapy as long as the polyps are small and asymptomatic. Regular sonographic controls are recommended since the risk of malignant transformation cannot be ruled out at present.

Keywords Peutz-Jeghers-syndrome Extraintestinal polyps Hamartoma Gallbladder polyps 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • T. Vogel
    • 1
  • V. Schumacher
    • 2
  • A. Saleh
    • 3
  • J. Trojan
    • 4
  • G. Möslein
    • 1
  • for the Deutsche
  • Peutz-Jeghers-Studiengruppe
  1. 1.Allgemein und Unfallchirurgie, Medizinische Einrichtungen, Heinrich-Heine-Universität, Moorenstrasse 5, 40225 Düsseldorf, Germany Tel.: +49-211-8117406 Fax: +49-211-8117406DE
  2. 2.Institut für Humangenetik, Medizinische Einrichtungen, Heinrich-Heine-Universität, Moorenstrasse 5, 40225 Düsseldorf, GermanyDE
  3. 3.Klinik für Radiologie, Medizinische Einrichtungen, Heinrich-Heine-Universität, Moorenstrasse 5, 40225 Düsseldorf, GermanyDE
  4. 4.2. Abteilung für Innere Medizin, Johann-Wolfgang-Goethe-Universität, Frankfurt a.M., GermanyDE

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