软组织透明细胞肉瘤的临床与病理学特点

Study on Clinical Feature and Pathology in Clear Cell Sarcoma of Soft Tissue

目的研究软组织透明细胞肉瘤的临床特性、病理学特征及预后。方法观察12例病理诊断为软组织透明细胞肉瘤患者的临床特性,从肿瘤细胞的细胞质、细胞核及通过免疫组化染色进行分析。结果软组织透明细胞肉瘤患者常为青、中年,病程平均1.5年,肿瘤好发于四肢的肌腱和腱膜;病理上,肿瘤细胞排列成条索或巢状,形态相对一致,胞浆透明或嗜酸性颗粒状,核仁明显,部分肿瘤含有黑色素;肿瘤S-100和HMB 45免疫染色阳性,阳性率分别为100%(12/12)和75%(9/12),其预后类似恶性程度较高的其它肉瘤。结论软组织透明细胞肉瘤某些特征类似恶性黑色素瘤,但具有不同的临床特性、形态学和生物学行为,为一明确的临床病理学实体。

Objective To study clinical feature, pathology and prognosis in clear cell sarcoma of soft tissue. Methods 12 cases, pathologically diagnosed as clear cell sarcoma of soft tissue, were observed from the morphological, immunohistochemical, and clinical angle. Results The patients with clear cell sarcoma of soft tissue were predominantly young or middle - aged, with the clinical course of 1.5 years on the average. The tumor was chiefly found on tendons and aponeuroses of the extremities. Histopathologically, the tumor cells were arranged in cluster or nest- like, with almost identified appearance, including clear or granular eosinophilic cytoplasm, prominent nucleoli, and melanin demonstrated in 7 cases. 100% （12/12） of the subjects were S- 100 protein immunostained of tumor while 75% （9/12） were HMB 45 immunostained. The prognosis was similar to those of other highly malignant sarcomas. Conclusion Clear cell sarcoma of soft tissue possessing different clinical, pathological feature and biological behavior is defined as a kind of clinical and pathological entity, though some of the morphological characteristics were in commom with melanoma.