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Angiomatöse Skelettläsionen im Kindesalter

Skeletal vascular lesions in childhood and adolescence

  • Schwerpunkt: Kinderpathologie
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Zusammenfassung

Gemäß aktueller nosologisch orientierter Klassifikation werden vaskuläre Läsionen unterteilt in Tumoren mit eigenständigem Proliferationspotenzial und unterschieden von Malformationen mit früher Manifestation und einem Wachstum, welches synchron mit dem des kindlichen Organismus verläuft [10, 16]. Diese neue Klassifikation gilt auch für vaskuläre Läsionen des Skeletts.

Etwa 30% aller vaskulären Läsionen des Skeletts manifestieren sich in den ersten beiden Lebensjahrzehnten, wobei Malformationen überwiegen. Sie werden Störungen des komplexen Ablaufes der Angiogenese zugeschrieben und können in einer Minderzahl hereditär vorkommen. Multiple kutane vaskuläre Malformationen konnten Mutationen des TIE2- und des Glomulingens zugeordnet werden.

Vaskuläre Malformationen mit Beteiligung mehrerer Skelettelemente können einen aggressiven Verlauf unter dem Bild einer massiven Osteolyse zeigen.

Unter den epitheloiden vaskulären Tumoren des Skelettes lassen sich auch bei jungen Patienten morphologisch benigne epitheloide Hämangiome von epitheloiden Hämangioendotheliomen als Entität mit niedrigem Malignitätspotenzial abgrenzen.

Abstract

Currently, vascular lesions are being classified either as tumors with inherent proliferative potential or as vascular malformations with early manifestation and growth commensurate with the growth of the child [10, 16]. This new classification is also applicable to skeletal vascular lesions.

30% of all skeletal vascular lesions manifest in the first two decades of life, with a preponderance of malformations. They are attributed to disturbances in the complex cascade of angiogenesis and a minority may be hereditary. Multiple cutaneous vascular malformations are associated with mutations of the TIE2- and Glomulin-Genes.

Vascular malformations with involvement of multiple skeletal elements may pursue an aggressive course and manifest as massive osteolysis.

Among the epithelioid vascular tumours of bone, also in young patients, benign epithelioid hemangioma can be distinguished on a morphological basis from epithelioid hemangioendothelioma as an entity with low malignant potential.

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Danksagung

Wir danken Herrn Prof. Dr. Gernot Jundt für wertvolle Diskussion und Unterstützung.

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Authors and Affiliations

  1. Institut für Pathologie der Universität Basel

    E. Bruder

  2. Department of Pathology, Children’s Hospital, Boston, USA

    H. Kozakewich

  3. Institut für Pathologie der Universität Basel, Schönbeinstraße 40, 4031, Basel, Schweiz

    E. Bruder

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  1. E. Bruder
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  2. H. Kozakewich
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Correspondence to E. Bruder.

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Bruder, E., Kozakewich, H. Angiomatöse Skelettläsionen im Kindesalter. Pathologe 25, 311–316 (2004). https://doi.org/10.1007/s00292-004-0698-1

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