肾血管平滑肌脂肪瘤自发性破裂(附23例报告)

目的 探讨肾血管平滑肌脂肪瘤(RAML)自发性破裂的临床表现、诊断和治疗。方法 肾血管平滑肌脂肪瘤自发破裂23例，临床表现突发腰腹疼痛者21例，腰部酸胀者2例；其中伴血尿3例、恶心呕吐2例、休克3例、发热1例。23例B超检查，确诊13例；CT检查21例，确诊16例。结果 术前确诊RAML者16例，术中冰冻切片确诊2例，误诊为肾癌者4例、肾感染性病变者1例。行肾切除10例，肾部分切除和肿瘤剜除术各6例，数字减影血管造影(DSA)加栓塞术1例。结论 RAML自发性破裂后常改变临床症状和影像学特征，导致误诊和肾切除，综合分析临床表现和影像学资料可以提高术前确诊率，术中冰冻切片可以避免不必要的肾切除。

Renal angiomyolipoma with spontaneous rupture(report of 23 cases)

Objective To investigate the clinical features,diagnosis and treatment of spontaneous rupture of renal angiomyolipoma (RAML). Methods A total of 23 cases of spontaneous rupture of renal angiomyolipoma were studied retrospectively.The main clinical manifestations were summarized as follows.Sudden and unexpected lumbar and abdominal pain occurred in 21 cases and lumbar swollen pain in 2.Of them 3 cases concomitantly had hematuria.Nausea and vomiting occurred in 2 cases,shock in 3 and fever in 1.All cases underwent B-ultrasonic examination and 21 had CT examination. Results Diagnosis of RAML was established preoperatively in 16 cases,but 4 cases were misdiagnosed for carcinoma of kidney,1 case for renal infectious disease.Two cases were diagnosed by frozen-section sample during operation.Nephrectomy was performed in 10 cases and nephron sparing nephrectomy in 12,and digital subtraction angiography (DSA) embolization in 1. Conclusions Spontaneously ruptured RAML often changes its clinical symptoms and imaging features,which may result in a high misdiagnosis rate and subsequent nephrectomy.Comprehensive analysis of clinical manifestations and image findings can improve preopreative diagnosis rate,and frozen slice sample examination during operation can avoid unnecessary nephrectomy.