The prevalence of RCC in TSC has been reported as 1.4%–4% in multiple studies.34 However, this is not dissimilar from the lifetime prevalence of RCC of 1.3%–2.3% in the general population of 1.3%.41 Reported TSC-associated RCC has been characterized as papillary, oncocytic chromophobe, or granular eosinophilic macrocystic variants.42,43 The risk of RCC seems to be higher in TSC2 mutants than TSC1, particularly for patients with TSC2/PKD1 contiguous gene syndrome. This evidence concerns the gene TSC1 and renal cell carcinoma.