软组织透明细胞肉瘤临床进展

Advances in clinical research on soft tissue clearcell sarcoma

透明细胞肉瘤是一种罕见的高度恶性软组织肉瘤。多发于青少年及中年,以四肢远端的深部软组织多见,常与肌腱和腱膜相毗邻,易复发和转移。组织来源尚未明确,限制了有效的预后因素分析及治疗原则的确立。早期诊断和首次广泛切除是改善透明细胞肉瘤预后的关键。肿瘤大小与是否发生转移关系密切,是影响预后的重要因素。本文就透明细胞肉瘤的临床病理特点、鉴别诊断、临床治疗及预后分析的进展进行综述。

Clear cell sarcoma （CCS） is a rare malignant tumor of soft tissue. It occurs preferentially in adolescents and young adults. CCS shows a predilection for the deep soft tissues of the lower extremities close to the tendon and aponeuroses which is associated with a high propensity of local recurrence and distant metastases. The histogeneous source is uncertain, which restrained the establishing of a practical prognosis system and an appropriate treatment strategy. Early diagnosis and extensive excision were crucial to improve the prognosis of clear cell sarcoma. The tumor size was related with metastasis, and it was a key factor on tumor prognosis. There was no supportive evidence on the therapeutic effectiveness of chemotherapy in the treatment of clear cell sarcoma. This review summarized the recent progress on the pathological characteristics, diagnosis, treatment and prognosis analysis of clear cell sarcoma.