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隆突性皮纤维肉瘤(dermatoflbrosarcoma protuberans,DFSP)是一种罕见的皮肤来源性肉瘤,呈侵袭性生长。主要以复发为主,转移罕见,如果发生远处转移,通常转移到肺,很少发生淋巴结转移。DFSP的主要治疗方式是根治性局部广泛切除,放射治疗作用有限,化疗基本上没有作用。伊马替尼为治疗实体肿瘤的一个典范,目前已证实伊马替尼在DFSP患者全身治疗中有效,并具有广泛的应用前景。作者就DFSP的治疗方式、分子发病机制以及伊马替尼治疗DFSP仍存在的问题进行综述。
Dermatoflbrosarcoma protuberans (DFSP) is a rare, skin-derived sarcoma that grows aggressively. Mainly to relapse-based, rare transfer, if the distant metastasis, usually transferred to the lungs, rare occurrence of lymph node metastasis. The main treatment of DFSP radical radical excision, limited role of radiation therapy, chemotherapy basically no effect. Imatinib is a model for the treatment of solid tumors. It has been confirmed that imatinib is effective in the systemic treatment of DFSP patients and has broad application prospects. The authors reviewed the treatment of DFSP, the molecular pathogenesis and the problems still existing in the treatment of DFSP with imatinib.