While AML and cysts are the most common renal manifestations in patients with inherited TSC syndromes, approximately 4% will develop renal cell carcinoma (RCC). These include RCC with clear cytoplasm, papillary architecture, and prominent smooth muscle stroma; RCC with granular eosinophilic cytoplasm and macrocystic architecture; and RCC resembling the eosinophilic variant of chromophobe RCC. In recent years and in five studies in the March 2022 issue of Modern Pathology, sporadic counterparts to the hereditary tuberous sclerosis complex-associated RCC that are associated with somatic TSC/MTOR pathway mutations have now been described.
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