60例肾脏血管平滑肌脂肪瘤临床病理分析

目的 探讨肾脏血管平滑肌脂肪瘤(renal angiomyolipoma，RAML)的临床病理特点、诊断、鉴别诊断以及免疫组织化学对RAML的诊断价值。方法 复习60例RAML的临床资料、影像学资料，采用石蜡切片进行脏染色及免疫组织化学染色。结果 60例：RAML中50例女性，10例男性，平均年龄43．8岁。40例有临床症状。6例合并结节性硬化症。大体检查多数肿瘤与周围组织分界清楚。光镜下由厚壁血管、梭形或上皮样平滑肌细胞及脂肪混合而成。免疫组织化学染色瘤细胞A103和HMB45、SMA均有良好表达。随访42例无肿瘤复发。结论 RAML的确诊依赖于临床、影像学资料及病理学三者相结合。A103有助于确诊。CT诊断困难者可行术前穿刺活检或术中冷冻切片检查并辅以免疫组织化学染色以指导医生确定治疗方案。

60 cases of renal angioleiomyolipoma a clinicopathologic analysis

Objective To investigate the clinicopathologic characteristics and differential diagnosis of renal angioleiomyolipoma(RAML) and to evaluate immunohistochemical reactivity in the diagnosis of RAML. Methods A retrospective study was done by reviewing the clinical and imaging documents of 60 cases of RAML. HE and immunohistochemical stainings were performed on paraffin-embeded tissues with a panel of antibodies including A103. Results Among the 60 cases (male /female=10 /50, mean age 43.8), 40 cases were symptomatic. 6 cases had associated with tuberous sclerosis. Most of tumors were sharply demarcated from the surrounding kidney parenchyma. Histologically, the tumors were composed of three components: thick-walled blood vessels, spindle or epithelioid smooth muscle cells and adipose tissues. Immunohistochemically, the tumor cells were strong positive for A103, HMB45 and smooth muscle actin(SMA). 42 patients were followed up and all were alive with no evidence of recurrence. Conclusion RAML is a rare mesenchymal tumor of kidney. Combination of clinical manifestation, imaging documents and pathologic study might be helpful to its correct diagnosis. A103 is a promising marker for pathologic diagnosis of RAML. If necessary, preoperative fine needle aspiration biopsy and midoperation frozen section study combined with immunohistochemical study should be undertaken to guide the treatment.