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基于组织学亚型的软组织肉瘤的医学治疗

[Medical treatment of soft tissue sarcomas based on the histological subtype].

作者信息

Pápai Zsuzsanna

机构信息

Onkológiai Osztály, MH-Egészségügyi Központ, Budapest, Hungary.

出版信息

Magy Onkol. 2014 Mar;58(1):53-8. Epub 2014 Feb 1.

Abstract

The medical treatment of adult soft tissue sarcomas is more and more dictated by the histological subtype, this applies to both cytotoxics and target therapies. Doxorubicin and ifosfamid are the two drugs used either in monotherapy or combination with the best established response rates in adult soft tissue sarcomas for several years. In addition to these compounds there is evidence of efficacy of new drugs such as taxanes in angiosarcoma, gemcitabine+taxanes combination in leiomyosarcomas, trabectedin in leiomyosarcomas and liposarcomas with an extremely high activity in myxoid liposarcoma. With regard to target therapy pazopanib seems especially active in leiomyosarcomas and synoviosarcomas, but totally inactive in liposarcomas, sunitinib and cediranib in alveolar soft part sarcomas, sunitinib and bevacizumab+temozolamide combination in solitary fibrous tumors, and sorafenib in angiosarcomas. mTOR inhibitors are active in PEComas (perivascular epitheloid cell tumors) and crizotinib in ALK rearranged inflammatory myofibroblastic tumors. The efficacy of imatinib and sunitinib in GIST tumors are established, and that of imatinib in dermatofibrosarcoma as well.

摘要

成人软组织肉瘤的医学治疗越来越取决于组织学亚型,这适用于细胞毒性药物和靶向治疗。多柔比星和异环磷酰胺是多年来在成人软组织肉瘤中用于单药治疗或联合治疗且有效率最高的两种药物。除了这些化合物外,还有证据表明新药具有疗效,如紫杉烷类药物治疗血管肉瘤、吉西他滨联合紫杉烷类药物治疗平滑肌肉瘤、曲贝替定治疗平滑肌肉瘤和脂肪肉瘤(在黏液样脂肪肉瘤中活性极高)。关于靶向治疗,帕唑帕尼似乎在平滑肌肉瘤和滑膜肉瘤中特别有效,但在脂肪肉瘤中完全无效;舒尼替尼和西地尼布用于治疗肺泡软组织肉瘤;舒尼替尼和贝伐单抗联合替莫唑胺用于治疗孤立性纤维性肿瘤;索拉非尼用于治疗血管肉瘤。mTOR抑制剂在PEComas(血管周上皮样细胞瘤)中具有活性,克唑替尼在ALK重排的炎性肌纤维母细胞瘤中具有活性。伊马替尼和舒尼替尼在胃肠道间质瘤中的疗效已得到证实,伊马替尼在皮肤纤维肉瘤中的疗效也已得到证实。

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