摘要:目的：探讨皮肤平滑肌肉瘤的临床病理特点和诊断要点及预后。方法:对2例皮肤平滑肌肉瘤组织病理学、免疫组化观察,并复习相关文献。结果: 例1为皮下平滑肌肉瘤,具有结节型的生长形态,瘤细胞丰富,异型性较大,核分裂活跃；例2为真皮平滑肌肉瘤,具有弥漫型的生长形态,瘤细胞较少,分化好,核分裂象不明显。免疫组化2例均表达SMA、MSA、Vim,1例灶性表达Desmin。2例随访迄今均无复发及转移。结论:皮肤平滑肌肉瘤少见,可分为真皮和皮下两种类型,两者具有不同的组织起源和预后特点,我们要注意区分,诊断除核分裂象计数外,尚需进行综合评估,对某些病例建议采用恶性潜能未定的平滑肌肉瘤的诊断,治疗首选外科手术切除。Abstract: Objective: To investigate the clinic pathological features diagnosis main point and prognosis of cutaneous leiomyosarcoma（CLMS）.Methods:Histopathology,immunohistochemical stainings observation were analyzed in two cases of CLMS and the related literatures were reviewed. Results:Case 1 was subcutaneous leiomyosarcoma with tubercular growth pattern,rich tumor cell,big heterogeneous type,active mitotic；Case 2 was dermis leiomyosarcoma with diffuse growth pattern,few tumor cell,well differentiated,no more mitotic. Immunohistochemically,the two cases reacted positively with smooth muscle action、MSA and Vim,Case 1 also expressed desman partially. The two cases were revisited to date,no recurrences and metastases.Conclusion:Cutaneous leiomyosar coma is a rare tumor,subdivided into dermis and subcutaneous forms because of their different tissue origins and prognosis features. We must discriminate between them. Diagnosis need synthetic appraisal besides mitotic counts and “smooth muscle tumor of uncertain malignant potential” should be used for diagnosis of certain cases.Primary treatment for cutaneous leiomyosarcoma is surgical excision.