Zusammenfassung
Das Dermatofibrosarcoma protuberans (DFSP) gehört zu den seltenen Tumoren der Haut, stellt aber gleichzeitig das häufigste kutane Sarkom dar. Es handelt sich um einen Tumor von fibroblastischer Differenzierung, der langsam, unterminierend und lokal destruierend wächst, aber nur selten metastasiert. Das klinische Bild ist insbesondere bei initialen Befunden oft uncharakteristisch, weshalb die Diagnose in der Regel erst spät und meist durch Biopsie und Histologie gestellt wird. Die Therapie der Wahl ist die komplette chirurgische Exzision des Tumors mit einem Sicherheitsabstand von 2–3 cm. Bei geringeren Sicherheitsabständen besteht ein erhöhtes Risiko für die Ausbildung von Lokalrezidiven. Inoperable oder metastasierte DFSP können bestrahlt werden; die Strahlensensitivität des DFSP gilt als hoch. Nachdem für ca. 90% aller DFSP eine charakteristische Chromosomentranslokation (17:22), einhergehend mit einer Genfusion, beschrieben wurde, die zur Ausbildung einer kontinuierlichen autokrinen Wachstumsstimulation über den PDGFβ-Signalweg führt, existiert die zusätzliche Möglichkeit einer molekular zielgerichteten Therapie. Die erste hierfür zugelassene Substanz ist der Multikinaseinhibitor Imatinib, der in klinischen Studien an ausgedehnten oder metastasierten DFSP eine Remissionsrate von ca. 70% zeigte.
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare tumor but still the most common cutaneous sarcoma. DFSP is a tumor of fibroblastic origin, characterized by a slow, undermining and locally destructive growth pattern, which only rarely metastasizes. The clinical appearance, especially of smaller lesions, is often not characteristic, so that diagnosis is often made late and only on biopsy findings. The standard treatment of DFSP is excision with safety margins of 2 to 3 cm. If smaller margins are employed, the risk of local relapse is high. Surgically incurable or metastatic DFSP can be irradiated; the cells are generally radiation-sensitive. 90% of DFSP carry a chromosome translocation of 17 and 22, harboring a gene fusion, which results in a continuous activation of the PDGFβ signal transduction pathway. This finding led the way to a new molecular targeted therapy of DFSP using inhibitors of the PDGFβ pathway. The first drug to be registered for targeted treatment of locally advanced or metastasized DFSP is the multikinase inhibitor imatinib, showing a response of about 70% in clinical trials.
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Der korrespondierende Autor weist auf folgende Beziehung hin: Referententätigkeit und Drittmittel: Novartis.
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Ugurel, S. Dermatofibrosarcoma protuberans. Hautarzt 59, 933–941 (2008). https://doi.org/10.1007/s00105-008-1501-7
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DOI: https://doi.org/10.1007/s00105-008-1501-7