Medical treatment of angiosarcomas.

PURPOSE OF REVIEW: Medical treatment of angiosarcomas is based on chemotherapy and tyrosine kinase inhibitors with limited efficacy. Immune therapy represents an exciting new therapeutic opportunity in this indication.

RECENT FINDINGS: Several clinicals trials, randomized or not, have been recently released to better define the scope and efficacy of immune therapy in angiosarcoma.

SUMMARY: Angiosarcoma is a rare and malignant aggressive soft tissue sarcoma with a wide diversity of presentation in terms of location, aetiology, biology. Medical treatment may be considered as neo adjuvant treatment for locally advanced disease or as palliative treatment in case of metastatic spreading. Doxorubicin and paclitaxel are the two regimens with highest level of evidence of efficacy. Other chemotherapy like gemcitabine or antiangiogenic tyrosine kinase inhibitors may also be efficient. Immune therapy represents an exciting new opportunity under assessment, the selection of patients likely to benefit from this strategy according to clinical or biological criteria remains to be defined. The access to innovative therapy for angiosarcoma is limited by the rarity and heterogeneity of the disease.