结节性硬化症合并双肾错构瘤1例报告并文献复习

Diagnosis and Treatment for Renal Angiomyolipoma Associated with Tuberous Sclerosis:One Case of Report and Review of the Literature

目的:探讨结节性硬化症(TSC)合并肾错构瘤(RAML)的临床表现、病理特点、治疗方法及预后。方法:分析1例TSC合并RAML患者的病历资料并复习相关文献。结果:患者左肾于7年前因"错构瘤破裂"行切除术,右肾TSC合并RAML采取保守治疗,待病情好转后出院,定期复查B超,随访至今,病情稳定。结论:结节硬化型肾错构瘤临床较为少见,对于影像学检查肿瘤无脂或少脂信号者,结合结节硬化症的典型临床表现,有助于和肾癌鉴别;治疗方案应根据肿瘤的大小、分布、发展情况及症状决定,对于短期肿瘤生长较快或者肿瘤内有坏死者,须考虑恶变的可能,应行肾切除。

Objective： To investigate the clinical and pathological characteristics, treatment and prognosis of RAMI. associated with TSC. Methods：The clinical data and pathological characteristics of a RAML patient associated with TSC diagnosed and treated in our hospital were analyzed and relevant literature was reviewed. Results： The patient received nephreetomy 7 years ago because of the AMI. rupture of the left kidney. During the hospital stay, the patient received conservative therapy and six-month follow-up of B-ultrasound showed no further development of the disease after she was discharged. Conclusions： RAMLwith TSC is a rare case. It should be diffrentiated from renal carcinoma when lipoma signal isn＇t detected in tumor. The treatment is based on tumor size,progression and associated symptoms. The tumor should be considered malignant if it develops too fast in a short period or necrosis is present in tumor.