title: [Clinical analysis of tuberous sclerosis complex complicated with renal angiomyolipoma: a report of 22 cases].
abstract: OBJECTIVE
  To explore the diagnosis and treatment of tuberous sclerosis complex complicated with renal angiomyolipoma.
  
  
  METHODS
  The clinical data of 22 patients with tuberous sclerosis complex complicated with renal angiomyolipoma were analyzed retrospectively.
  
  
  RESULTS
  There were 12 males and 10 females with a mean age of 23 (1-46) years. All of them had bilateral multiple renal angiomyolipomas. The mean tumor size was 8.5 (0.7-18.0) cm in diameter. The presence or absence of clinical symptoms showed a significant correlation with tumor size.Eight patients with angiomyolipoma under 4 cm in diameter were continuously monitored at an outpatient clinic. There were 8 patients with lesions of 4-10 cm.Five of them underwent nephron-sparing surgeries and another 3 monitored at an outpatient clinic. There were 6 patients with tumor over 10 cm. Three of them underwent transcatheter arterial embolization and one case took rapamycin after embolization.One patient with concurrent renal cell carcinoma underwent partial nephrectomy. Chronic renal failure occurred in one case dying of pulmonary lymphangiomyomatosis with serious hemoptysis. During a mean follow-up of 25 months, neither severe renal hemorrhage nor symptomatic aggravation was found.In the case of rapamycin, there was 10% reduction in the size of angiomyolipoma.
  
  
  CONCLUSIONS
  Most cases of tuberous sclerosis complex are complicated with bilateral multiple renal angiomyolipoma. The small lesions under 4 cm in diameter may be monitored at an outpatient clinic.For those larger (>4 cm) or symptomatic ones, medication, embolization or surgery is necessary.
authors.affiliation: Department of Urology, Peking Union Medical College Hospital & Chinese Academy of Medical Sciences, Beijing 100730, China.
authors.forename: Wen-yun
authors.initials: WY
authors.lastname: Ting
authors.forename: Yu-shi
authors.initials: YS
authors.lastname: Zhang
authors.forename: Han-zhong
authors.initials: HZ
authors.lastname: Li
authors.forename: Xue-bin
authors.initials: XB
authors.lastname: Zhang
authors.forename: Wei-feng
authors.initials: WF
authors.lastname: Xu
mesh_terms: D000293:Adolescent; D000328:Adult; D018207:Angiomyolipoma / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D055815:Young Adult