平滑肌肉瘤的最新研究进展

The recent research findings in Leiomyosarcoma

平滑肌肉瘤(LMS)是起源于平滑肌细胞的间质性恶性肿瘤。发病机制仍不清楚。不同类型的LMS存在染色体核型异常。在LMS可观察到RB1和PTEN表达异常可激活细胞周期和PI3K/AKT信号通路。手术完全切除是治愈本病的惟一方法,不能手术切除的转移性LMS无法治愈。全身性化疗始终是姑息治疗。LMS对联合应用吉西他滨和多西他赛治疗敏感,目前成为本病的标准治疗方案。采用曲贝化疗对在LMS有效,可以使疾病长期稳定。帕唑帕尼是最近批准用于治疗LMS的一种口服多激酶抑制剂。对不同类型的LMS,目前迫切需要进一步了解其生物学特性、提高诊断水平及开发有效和毒性低的治疗药物。

Leiomyosarcoma （LMS） is a mesenchymal malignancy derived from the smooth muscle cell. The mechanisms of genesis are still unknown. The karyotypic defects are existed in the different subtypes of leiomyosarcoma. Abnormal cell cycle through RB 1 and P13K/AKT pathway activation are observed in leiomyosarcoma. Patients with unresectable metastatic leiomyosarcoma are incurable and the chemotherapy for systemic disease is always palliative. The only curative option in LMS is surgery. LMS is sensitive to the combination of gemcitabine and docetaxel regimen, which is currently considered a standard of treatment in those patients. Chemotherapy with trabectedin has shown exquisite activity in leiomyosarcoma, mainly in the form of long disease stabilization. Pazopanib is an oral multikinase inhibitor recently approved for the treatment of leiomyosarcoma. Better understanding of the underlying biology of the LMS variants, improved diagnostics and more effective, less toxic therapeutic agents are required.