摘要Tuberous sclerosis complex(TSC)is an autosomal dominant disease caused by inactivating germline mutations in TSC1 or TSC2.These genes code for hamartin and tuberin,pro-teins that normally form a complex that down-regulates the mechanistic(or mammalian)target of rapamycin(mTOR)pathway,which is crucial to cell growth,proliferation and survival.The phenotypic effects of TSC include abnormali-ties in the brain,kidneys,skin,heart and lungs.Renal mani-festations include cyst formation and the development of tumors,especially multiple benign tumors called angiomy-olipomas(AMLs)as well as renal cell carcinoma(RCC)and oncocytoma[1].Almost all renal masses in children with TSC are AMLs,and these can often be confidently diag-nosed by imaging,most often by the identification of sig-nificant fatty components on computed tomography(CT)or magnetic resonance imaging(MRI).Occasionally,however,even a large AML will contain no identifiable fatty elements.