Zusammenfassung
Das Angiosarkom der Haut ist ein hochmaligner Endothelzelltumor mit hoher Mortalität, der einer zuverlässigen Diagnose bedarf. Aufgrund der morphologischen Vielfalt des Angiosarkoms und seiner breiten Differenzialdiagnose kann dies jedoch ernsthafte Probleme bereiten. In vielen Fällen beruht die Diagnose auf dem immunhistochemischen Nachweis der Endothelzelldifferenzierung, der sich wegen mangelnder Sensitivität und Spezifität herkömmlicher Endothelzellantikörper als schwierig erweisen kann. Der folgende Artikel behandelt das klinische und histologische Spektrum des kutanen Angiosarkoms und hebt neue Erkenntnisse im Bereich der immunphänotypischen und molekulargenetischen Eigenschaften insbesondere in Bezug auf die relevante Differenzialdiagnose hervor.
Abstract
Cutaneous angiosarcoma is an aggressive endothelial cell neoplasm with a high associated mortality. Reliable and confident diagnosis is necessary but frequently challenging due to the wide morphological spectrum and broad differential diagnosis of the disease. Furthermore, the final diagnosis often relies on immunohistochemical evidence of endothelial cell differentiation which is further complicated by the lack of sensitivity and specificity of conventional endothelial cell markers. The following article outlines the clinical and histological spectrum of cutaneous angiosarcoma with emphasis on novel findings relating to immunohistochemistry and molecular genetics in the context of the relevant differential diagnosis.
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Danksagung
Ich möchte mich hiermit herzlichst bei Dr. Jason L. Hornick, Brigham and Women’s Hospital and Harvard Medical School, für die immunhistochemische Färbung für MYC (Abbildung 5f) bedanken.
Einhaltung ethischer Richtlinien
Interessenkonflikt. T. Brenn gibt an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.
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Brenn, T. Angiosarkome der Haut. Pathologe 36, 70–78 (2015). https://doi.org/10.1007/s00292-014-2002-3
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DOI: https://doi.org/10.1007/s00292-014-2002-3
Schlüsselwörter
- Differenzialdiagnose
- Endothelzelldifferenzierung
- Immunphänotypische Eigenschaften
- Molekulargenetische Merkmale
- ERG-Antikörper