Angiosarcoma: State of the art and perspectives

作者:Penel Nicolas*; Marreaud Sandrine; Robin Yves Marie; Hohenberger Peter
来源:Critical Reviews in Oncology/Hematology, 2011, 80(2): 257-263.
DOI:10.1016/j.critrevonc.2010.10.007

摘要

We propose a literature review of available data on angiosarcoma (AS). AS account for 1% of adult soft tissue sarcoma. Two risk factors are well-establish chronic lymhoedema, previous radiotherapy. Clinical presentations of AS are heterogeneous. Large resection followed, if possible, by adjuvant radiotherapy is the cornerstone of curative intent treatment of localized forms. There are no convincing data supporting the administration of adjuvant chemotherapy. For metastatic or locally advanced AS, doxorubicin and weekly paclitaxel seem to provide the longer progression-free survival. Three phase II or parts of phase 11 trials have been published in the last 2 years, investigating weekly paclitaxel, sorafenib and imatinib, demonstrating that clinical trials are feasible for such rare diseases. Biological evidences for the key role of angiogentic factors have been accumulated during the last years and support the further investigation of anti-angiogenetic agents alone and almost combination with chemotherapy in such disease.

  • 出版日期2011-11