结节性硬化症合并肾透明细胞癌的超声表现及文献复习

The sonographic manifestation of yuberous sclerosis complex with rare giant kidney cancer and review of the Literature

目的探讨结节性硬化症(tuberous sclerosis complex,TSC)合并肾透明细胞癌的超声诊断特征。方法对1例TSC合并肾透明细胞癌患者的超声资料及临床表现进行回顾性分析并相关文献复习。结果该例患者行肾肿瘤及肾脏根治性切除术,病理诊断肾透明细胞癌。随访6月未发现肿瘤复发及转移。结论 TSC合并肾透明细胞癌临床罕见。TSC合并肾脏巨大肿块并坏死者,超声表现为囊实性混合回声并多处钙化,瘤周可见假包膜的丰富血流环绕征象,应高度考虑肿瘤恶变可能。结合超声造影的快进慢退模式可以对该病做出诊断并及早行肿瘤及肾脏根治性切除。

Objective To explore the characteristics of ultrasonography of tuberous sclerosis complex with renal clear cell carcinoma.Methods The clinical and imaging data in the case of tuberous sclerosis complex with renal clear cell carcinoma were retrospectively analyzed and the relevant literature was reviewed.Results The patient received renal cancer radical nephrectomy.The pathological diagnosis was renal clear cell carcinoma.There was no tumor recurrence or distant metastasis within 6-month follow-up.Conclusion The tuberous sclerosis complex complicated with renal cell carcinoma is rarely reported.The mixed ultrasonic presence of cystic or solid echo with several calcification,and tumor periphery visible psuedocapsule encompassed with blood flow indicated the sign of renal cancer.The ultrasonic imaging may provide the early diagnostic rationale of the disease and early and kidney tumor resection.