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Leiomyomatose des Rektums

Fallbericht und Literaturübersicht

Leiomyomatosis of the colon: case report and literature review

  • Schwerpunkt: Gastrointestinale Motilitätsstörungen
  • Published:
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Zusammenfassung

Vorgestellt wird der bei unserem 67-jährigen männlichen Patienten im Rektum lokalisierte 5. Fall einer intestinalen Leiomyomatose. Dabei handelt es sich um eine manschettenartig intramural und teilweise auch extramural entwickelte und längerstreckig stenosierende tumoröse oder tumorartige glattmuskuläre Proliferation der Darmwand. Bei unserem Patienten musste nach über 5 Jahre bestehender massiver obstruktiver Symptomatik eine Rektumamputation durchgeführt werden. Histologisch und immunhistologisch fanden sich gleichartige Befunde wie bei Leiomyomen. Ein Merkmal der Leiomyomatose scheint jedoch das Auftreten von für Leiomyome untypischen hyalinisierenden Gefäßwandveränderungen zu sein. Eine weitere Besonderheit unseres Falls bestand im Nachweis von sonst nur bei gastrointestinalen Stromatumoren zu beobachtenden „skeinoid fibers“.

Abstract

We present the findings of a 67 year old male patient with an intestinal leiomyomatosis localized in the rectum. To our knowledge, this is the fifth case of intestinal leiomyomatosis reported so far. The most characteristic findings of this rare disease include a cuff-like tumorous proliferation of smooth muscle within the bowel wall which may extend into extramural tissue and result in a stenosis of a longer bowel segment. Because of severe obstructive symptoms over 5 years, the patient had to undergo surgery with resection of the rectum. The histological examination revealed a morphology and immunophenotype comparable to usual leiomyomas with the exception of hyalinosis-like changes in the blood vessels, apparently a special feature of leiomyomatosis. A novel finding in our case was the occurrence of skeinoid fibers which have so far only been reported in gastrointestinal stromal tumors.

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Notes

  1. Hinzuweisen ist darauf, dass der Terminus Leiomyomatosis in einigen Kasuistiken auch für multipel vorkommende nicht konfluierende, d. h. nicht das typische Bild einer flächigen Tumormasse bietende intestinale Leiomyome [4, 11] verwendet wurde.

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Danksagung

Die Autoren danken Herrn Norbert Wey für die Anfertigung der Abbildungen.

Interessenkonflikt

Die korrespondierende Autorin versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen.

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Authors and Affiliations

  1. Institut für Pathologie und Rechtsmedizin, Kantonsspital Chur, Loestraße 170, 7000, Chur, Schweiz

    B.-C. Padberg & M. Germer

  2. Chirurgische Abteilung, Israelitisches Krankenhaus Hamburg, Hamburg, Deutschland

    A. Emmermann & C. Zornig

  3. Gemeinschaftspraxis für Pathologie, Hamburg, Deutschland

    S. Schröder

Authors
  1. B.-C. Padberg
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  2. A. Emmermann
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  3. C. Zornig
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  4. M. Germer
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  5. S. Schröder
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Corresponding author

Correspondence to B.-C. Padberg.

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Widmung

Herrn Prof. Dr. Gerhard Seifert, emeritierter Ordinarius für Pathologie der Universität Hamburg, aus Anlass seines 85. Geburtstags gewidmet.

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Padberg, BC., Emmermann, A., Zornig, C. et al. Leiomyomatose des Rektums. Pathologe 28, 161–165 (2007). https://doi.org/10.1007/s00292-007-0898-6

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  • DOI: https://doi.org/10.1007/s00292-007-0898-6

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