摘要
目的 了解透明细胞肉瘤的病理与临床特点。方法 复习中国医学科学院肿瘤医院1973 年至1998 年收治的6 个病例及复习文献,对其病理及临床特点、治疗方法及疗效、预后因素等做一总结。结果 透明细胞肉瘤来源于原始神经外胚层细胞,由在胚胎形成过程中迷走于肌腱和肌筋膜中的神经脊细胞发展而来,具有特征性的染色体改变,即t(12;22)(q1314;q1213) 等;病变与肌腱及肌筋膜关系密切;易出现区域淋巴结转移;手术切除后易局部复发。治疗原则应为手术加术后大野放射治疗并采用多次缩野技术,区域淋巴结应给予手术清扫或放射治疗。5 年生存率为37 % ~67% 。影响预后的因素有肿瘤大小及是否伴有坏死。结论 透明细胞肉瘤是一类有自身特征的肿瘤。
Objective To study the clinicopathologic characteristics of clear cell sarcoma.Methods Six patients with clear cell sarcoma admitted into our hospital from 1973 to 1998 were analyzed with review of literature. The clinicopathologic features, treatment principle, prognosis and prognostic factors of clear cell sarcoma were summarized.Results Clear cell sarcoma represents a malignant neuroectodermal tumor derived from cells that have migrated from the neural crest during embryonic life. It has characteristic aberrations of chromosome, mainly t(12;22)(q13 14;q12 13) and is closely associated with tendons and aponeuroses. Regional lymph node metastasis is common. It often relapses locally if the tumor is treated by surgery alone. The treatment principle must be surgery plus postoperative radiotherapy with the field large enough. Cone down technic is indicated twice during the treatment.The regional lymph nodes should be treated by surgical resection or radiotherapy giving a 5 year survival of 37%~67%. The prognosis is influenced by the tumor size and whether there is necrosis within the tumor. Conclusions The clear cell sarcoma is a kind of soft tissue sarcoma with inherent features. The treatment principle should be different from the other soft tissue sarcomas.
出处
《中华放射肿瘤学杂志》
CSCD
北大核心
1999年第4期225-228,共4页
Chinese Journal of Radiation Oncology
关键词
透明细胞肉瘤
病理学
治疗
预后
Clear cell sarcoma/pathology
Clear cell sarcoma/therapy
Prognosis