Intraglomerular Lesions of Renal Angiomyolipoma in Tuberous Sclerosis Complex: A Case Report

The association between tuberous sclerosis complex (TSC) and renal angiomyolipomas (RAML) is widely recognized, but the evidence of intraglomerular microlesions of angiomyolipoma in TSC is extremely rare. We report a 36-year-old woman with TSC, presenting as gross hematuria and hypovolemic shock. The patient was diagnosed with bilateral RAML and spontaneous rupture of right RAML. She underwent right nephrectomy. Microscopic examination revealed multiple bilateral RAML composed of dysmorphic blood vessels, smooth muscle, epithelioid cells and adipose tissue, as well as intraglomerular microlesions of these components in continuity with the glomerular basement membrane but without adhesion to the Bowman’s capsule. In this report we describe the histopathologic features and immunohistochemical markers of TSC-associated RAML with multiple parenquimatous lesions and isolated micro foci in the glomerular tuft. We also review recent discoveries related to possible pathogenesis of renal and intraglomerular lesions.

Keywords: angiomyolipoma, kidney, intraglomerular, Wunderlich’s syndrome.