不典型肾血管平滑肌脂肪瘤的诊治

目的：探讨不典型肾血管平滑肌脂肪瘤（RAML）的误诊原因，提高诊断率。方法：回顾性总结12例不典型RAML临床和影像学特点。结果：CT诊断正确率58．3％。B超检查诊断正确率50％。所有肿瘤都为单侧发病，肿瘤直径3～8cm，平均为5．5cm。5例患者行根治性肾切除术，4例患者行部分肾切除术，3例患者行肿瘤剜除术，术中冰冻切片提示肾错构瘤。其中3例患者伴有瘤内出血，肿瘤直径均大于6cm。术后病理均为RAML。结论：对不典型RAML术前做出正确诊断非常重要。应结合病史厦影象学检查，提高该病的诊断率，减少不必要的肾切除。

Clinical Experience of the Diagnosis and Treatment in Patients with Atypical Renal Angioleiomyolipoma

Objective:To investigate the misdiagnosis reason of renal angioleiomyolipoma(RAML).Methods:The clinical and imaging materials of 12 cases of RAML were reviewed retrospectively.Results:The accurate diagnostic rate of CT was 58.3%.The accurate rate of B-type ultrasound was 50%.The RAML all happened on the single side.The diameters of tumor were from 3 cm to 8 cm,and the average value was 5.5 cm.Among these patients,radical nephrectomy were performed in 5 patients,patial nephrectomy in 4 patients,tumor enucleation in 3 patients and the frozen sections of these tumors dis- played renal hamartoma.Hemorrage happened in 3 patients whose tumor diameters were larger than 6 cm.And all the final pathology displayed RAML.Conclusion:It's very important to make a correct diagnosis of atypical RAML before operation.Be familiar with the case history and imaging chacteristics of RAML will elevate the diagnostic rate and reduce the possibility of unnecessary nephrectomy.