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Seltene mesenchymale Läsionen bei Geschwisterkindern

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Zusammenfassung

Wir berichten über einen 15 Monate alten Jungen mit einem juvenilen aktiven ossifizierenden Fibrom in der rechten Nasenhaupt- und Nebenhöhle sowie über das 9 Monate alte Geschwister mit einem mesenchymalen Hamartom der Thoraxwand. Die Veränderungen zeigen zahlreiche Gemeinsamkeiten. In beiden Fällen handelt es sich um Veränderungen mit typischem Auftreten bereits bei der Geburt oder im frühen Kindesalter, die aufgrund der Kompression der angrenzenden Strukturen klinische Symptome hervorrufen. Beide Läsionen haben typischerweise ein mesenchymales Grundgerüst mit geringer Kernpleomorphie und herdförmiger Knochenbildung. Es handelt sich um tumorartige Prozesse des ortständigen Gewebes, ohne erhöhte Proliferationsrate. Diese Merkmale wären gut vereinbar mit hamartomatösen Läsionen.

Abstract

We report on a 15-month-old boy presenting a juvenile active ossifying fibroma in the right nasal cavity and the sibling, a 9-month-old girl with a mesenchymal hamartoma of the chest wall. The two lesions showed many similarities. Both lesions are present at the time of birth or in early life with local obstructive or compressive effects. The lesions have a similar mixture of mature and immature mesenchymal tissue with areas of ossification. The entities present a tumor-like development with an abnormal mixture of tissue indigenous to the specific area of the body without notable atypical cytologic features. These features are typical criteria for hamartoma lesions.

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Authors and Affiliations

  1. Abteilung Paidopathologie und Placentologie, Institut für Pathologie, Charité, Campus Virchow-Klinikum, Medizinische Fakultät der Humboldt-Universität, Berlin

    M. Guschmann

  2. Institut für Pathologie, Klinikum Benjamin Franklin, Freie Universität, Berlin

    J. Frege

  3. Klinik für Kinderchirurgie, Charité, Humboldt Universität, Berlin

    E. Lübbert & S. Golla

  4. Institut für Pathologie, Charité, Humboldt Universität, Berlin

    B. Rudolph

  5. Klinik für Hals-Nasen-Ohrenheilkunde, Charité, Humboldt Universität, Berlin

    K. Haake

  6. Abteilung für Pädiatrische Radiologie, Charité, Humboldt Universität, Berlin

    B. Stöver

  7. Abteilung Paidopathologie und Placentologie, Institut für Pathologie, Charité, Campus Virchow-Klinikum, Medizinische Fakultät der Humboldt-Universität, Augustenburger Platz 1, 13353, Berlin

    M. Guschmann

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  1. M. Guschmann
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  2. J. Frege
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  3. E. Lübbert
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  4. S. Golla
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  7. B. Stöver
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Correspondence to M. Guschmann.

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Guschmann, M., Frege, J., Lübbert, E. et al. Seltene mesenchymale Läsionen bei Geschwisterkindern. Pathologe 24, 220–225 (2003). https://doi.org/10.1007/s00292-002-0591-8

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  • DOI: https://doi.org/10.1007/s00292-002-0591-8

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