滑膜肉瘤的研究进展  

The reaserch progress in synovial sarcoma

作　　者：刘平平[1] 张兵林[1] 笪冀平[1] 

机构地区：[1]中日友好医院病理科,北京100029

出　　处：《中国组织化学与细胞化学杂志》2016年第3期280-284,共5页Chinese Journal of Histochemistry and Cytochemistry

摘　　要：滑膜肉瘤是一种伴有部分上皮分化的软组织肉瘤,是一种高级别肉瘤,可发生于各个年龄段和多个部位,常见于年轻人膝关节附近的软组织。90%以上滑膜肉瘤有特异性的染色体易位并形成SS18-SSX融合基因。滑膜肉瘤最重要的组织学形态特征是在梭形细胞肉瘤的基础上,出现局灶上皮分化,可表现为组织形态上的腺管或实性上皮巢,或仅在免疫组织化学或超微结构上检测到。由于滑膜肉瘤的预后、对化疗的反应与其它软组织肉瘤不同以及未来潜在的靶向治疗可能,滑膜肉瘤的病理诊断临床意义重大。本文复习了滑膜肉瘤相关文献,着重从滑膜肉瘤的临床特征与病理诊断、最新的免疫组织化学与分子遗传学发现、预后与治疗等方面归纳总结。Synovial sarcoma（SS） is a malignant mesenchymal neoplasm with variable epithelial differentiation,with a propensity to occur in the soft tissue around the knee of young adults,which can arise at almost any age and any anatomic site.It is generally viewed and treated as a high-grade sarcoma.It has been detected in more than 90%of SS the presence of a specific chromosomal translocation leading to the production of the SS18-SSX fusion oncogene,and the translocation remains unique to this tumor.Synovial sarcoma has a variety of morphologic patterns,but its chief forms are the classic biphasic pattern of glandular or solid epithelial structures with monomorphic spindle cells,and the monophasic pattern of fascicles of spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation.Since the chemosensitivity and prognostication of SS are different from other sarcomas,and because of its potential treatment with specific targeted therapies in the near future,correct pathological diagnosis is of great clinical significance.This review of SS summarizes research progress with emphasis on the diagnostic spectrum,recent immunohistochemical and genetic findings,and the differential diagnosis.

关 键 词：滑膜肉瘤 SS18-SSX 染色体易位 

分 类 号：R738.6[医药卫生—肿瘤]