[Angiosarcoma]

Angiosarcomas are rare tumors accounting for 1-2% of all soft tissue sarcomas. Nonetheless a dermatologist needs to be knowledgeable about this tumor because the prognosis is particularly poor. Angiosarcoma favors superficial soft tissues and skin (60%) with a clear predilection for the head and neck region. The average age of the patients presenting with cutaneous angiosarcomas is around 70 years with a peak incidence in the 8th decade. However, some subtypes may occur in children and adolescents. Secondary angiosarcoma after tissue-conserving radiation therapy for carcinoma of the breast represents an increasing problem, both the differentiation between atypical vascular lesions and true aggressive angiosarcoma and the therapy are challenging. The prognosis for angiosarcoma patients is gloomy despite all therapeutic efforts. Only early therapy seems to influence the outcome at all. There are some established guidelines for the primary and palliative therapy. Interesting new options of biomodulatory and molecularly targeted therapy can be envisioned.