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Tracheopathia osteoplastica

Ein 100-jähriges Mysterium

Tracheopathia osteoplastica

A 100-year-old mystery

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Zusammenfassung

Die Tracheopathia osteoplastica ist eine seltene Erkrankung der großen Atemwege mit bislang unbekannter Genese, die durch Ausbildung unter der Mukosa liegender verknöcherter Knoten gekennzeichnet ist. Die klinischen Symptome sind unspezifisch und in den meisten Fällen durch die krankheitsbedingte Tracheal- bzw. Bronchialstenose ausgelöst. Um die Histologie dieser Krankheit besser verstehen zu können, wurde ein kleines Kollektiv aus Trachealbiopsien (4 Fälle) und Sektionsfällen (16 Fälle) erstellt. Die untersuchten Trachealbiopsien und Obduktionsmaterialien zeigten zwei Formen der Verknöcherung der Trachea: zum einen die Tracheopathia osteoplastica tuberosa – die knotenbildende Form – und zum anderen die Tracheopathia osteoplastica peripherica – die praktisch nur im Obduktionsgut diagnostiziert werden kann. Die histologischen Befunde weisen darauf hin, dass die Tracheopathia osteoplastica zu den sklerosierenden Tracheopathien gehört, wobei der degenerative Fokus, von dem der Prozess ausgeht, sich an einem ungewöhnlichen Ort (in der Mitte des Knorpelrings) befindet. Eine Knorpelneubildung konnte in keinem Fall beobachtet werden.

Abstract

Tracheopathia osteoplastica is a very rare disease of the tracheobronchial tree with unknown origin and etiopathogenesis. The characteristic features are intramucosal nodules in the tracheal and/or bronchial mucosa. The clinical symptoms are unspecific and in most of the cases are consequences of airway stenosis. A collective of patient cases (4 tracheobronchial biopsies and 16 autopsy cases) was established to study the histology of tracheopathia osteoplastica and consisted of surgical pathology specimens (biopsies) and autopsy material. The study revealed two different types of tracheal ossification and nodule formation: (1) degenerative changes with nodule formation and ossification (tracheopathia osteoplastica tuberosa) and (2) diffuse degeneration of the tracheal cartilage with ossification of the outer third of the cartilaginous rings (tracheopathia osteoplastica peripherica). The histological changes indicate that tracheopathia osteoplastica is a type of sclerosing tracheopathy but the degenerative focus is at an unusual place (in the middle of the cartilage). No type of new cartilage formation was found in any of the cases.

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  1. Pathologisches Institut, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 220/221, 69120, Heidelberg, Deutschland

    C. Toth

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Toth, C. Tracheopathia osteoplastica. Pathologe 33, 129–134 (2012). https://doi.org/10.1007/s00292-011-1561-9

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