Bilateral multiple renal angiomyolipomas associated with tuberous sclerosis (report of 4 cases) / 中华泌尿外科杂志

ABSTRACT

Objective To study the clinical features of renal angiomyolipomas with tuberous sclerosis and to improve the recognization of this disease. Methods The diagnosis and treatment of 4 cases of tuberous sclerosis with bilateral and multiple renal angiomyolipomas were reviewed.The age range was 17 to 44 years,two of them were men.One patient presented with the classic triadfacial angiofibroma,seizures,and mental retardation.Facial angiofibroma occurred in three and seizures were found in two,three patients showed multiple extrarenal organ involvement including liver,spleen and bone.Brain CT revealed classical calcified cortical tuber and(or) subependymal nodule in three cases. Results The diagnosis of renal angiomyolipomas in three cases was affirmed on CT scan.However,the giant renal angiomyolipomas in one case can not be diagnosed from renal CT scan which did not demonstrate characteristic fatty tissue whereas the diagonsis was confirmed by ultrasound-guided right renal tumor biopsy and subsequent right nephrectomy for the large leisions.Regular monitoring of one patient was adopted for his mild symptomatic small renal leisions,the other two with hemorrhage in renal angiomyolipomas were reluctant to aggressive treatment and underwent conservative therapy.Follow-up ranged 6 to 36 months, none had recurrent symptoms and no significant growth of renal lesions was observed. Conclusions CT may not be pathognomonic for renal angiomyolipomas associated with tuberous sclerosis,needle biopsy may be needed to exclude renal carcinoma.The choice of treatment is based on tumor size distribution pattern,progression,and associated symptoms.