Angiosarcomas are a rare subtype representing 1-2% of
soft tissue sarcomas. Risk factors are rarely elucidated but
radiotherapy and
lymphedema are the most common ones, usually following local treatment for local
breast cancer. Despite the improvement of our knowledge, the prognosis remains poor with 35-40% of 5 year-overall survival. Local treatment when feasible should include a R0 surgery completed with adjuvant radiation. When metastatic, front lines
chemotherapies include doxorubicine or weekly
paclitaxel. If possible, in oligometastatic patients,
metastasectomy should always be considered allowing the best responses. The knowledge of
angiosarcoma's biology is rapidly increasing and new
biomarkers are emerging. The use of
immunotherapy in particular subtypes including head and neck
angiosarcomas shows promising results. The model of the
angiosarcoma project, a patient-participating study, seems to be an excellent way to study rare
tumors. We should focus our efforts on understanding the underlying molecular biology to propose the best
precision medicine for those patients.