EORTC Headquarters, Belgium.
Crit Rev Oncol Hematol. 2011 Nov;80(2):257-63. doi: 10.1016/j.critrevonc.2010.10.007. Epub 2010 Nov 4.
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 II 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.
我们对现有的血管肉瘤（AS）相关文献进行了回顾。AS 占成人软组织肉瘤的 1%。两种明确的危险因素是慢性淋巴水肿和既往放疗。AS 的临床表现存在异质性。对于局限性疾病，根治性治疗的基石是尽可能进行大切除，然后辅助放疗。没有令人信服的数据支持辅助化疗的应用。对于转移性或局部晚期 AS，多柔比星和每周紫杉醇似乎可提供更长的无进展生存期。在过去的 2 年中，已经发表了三项 II 期或部分 II 期试验，研究了每周紫杉醇、索拉非尼和伊马替尼，表明对于此类罕见疾病，临床试验是可行的。近年来，血管生成因子在其中的关键作用的生物学证据不断积累，支持进一步研究单独使用抗血管生成药物，以及几乎与化疗联合应用于此类疾病。
