Diagnosis and treatment of bilateral multiple renal angiomyolipomas associated with tuberous sclerosis complex
10.3760/cma.j.issn.1000-6702.2011.12.012
- VernacularTitle:双肾多发性血管平滑肌脂肪瘤合并结节性硬化症的诊治分析
- Author:
Jun ZHOU
;
Chaozhao LIANG
;
Yuanping YE
;
Ming LI
;
Xiansheng ZHANG
;
Zongyao HAO
;
Yifei ZHANG
;
Song FAN
;
Changqin JIANG
- Publication Type:Journal Article
- Keywords:
Tuberous sclerosis;
Kidney neoplasms;
Angiomyolipoma
- From:
Chinese Journal of Urology
2011;32(12):819-822
- CountryChina
- Language:Chinese
-
Abstract:
Objective To discuss the clinical features of bilateral multiple renal angiomyolipomas (RAML) with tuberous sclerosis complex (TSC).Methods Clinical data of 10 cases of bilateral multiple RAML associated with TSC treated from Jan.2003 to Dec.2010 were retrospectively reviewed.There were 4 males and 6 females in the study group,aged from 18 to 45 years,with an average age of 34 years.Three cases were treated urgently due to RAML hemorrhage.Five cases were treated due to back pain.One case was treated for hematuria.One case was diagnosed during physical examination.Facial angiofibroma occurred in 7 cases and facial flat speckle in 1 case.Epilepsy was indentified in 6 cases.Four cases had hypophrenia and 2 cases presented with symptoms of the classic triad:epilepsy,facial angiofibroma and hypophrenia.One case underwent urgent selective transcatheter arterial embolization (SEA).Eight cases (9 sides) underwent renal sparing operation.Conservative therapy was adopted in 2 cases.ResultsThe SEA was done successfully and bleeding ceased.AⅡ surgeries were completed successfully.The diameter of the biggest tumor resected was 8.0 cm.The number of tumors resected was 2 - 6,with an average of 3.5.The mean operative time was 70 min (range 50 to 120 min).The mean time of controlling the renal artery was 26 min.The mean intraoperative hemorrhage was 150 ml.One case required a blood transfusion.None of the cases re-hemorrhaged after operation.Pathology revealed RAML.There was no recurrence in 8 cases and follow-up ranged from 3 to 60 months,with an average of 25 months.A hemorrhage did occur on the oppositer side in 1 case 4 months after surgery.In 2 cases,a conservative therapy of regular monitoring and follow-up at 24 and 36 months was chosen.The serum creatinine of all the patients was in a normal range.Conclusions The bilateral multiple RAML associated with TSC must be diagnosed as soon as possible and treated depending on the size,location and developing circumstance of the tumor in a timely fashion.Renal sparing surgery and SEA are effective therapies.
