Recent additions of succinate dehydrogenase (SDH) and (Hereditary Leiomyomatosis and Renal Cell Cancer, HLRCC, associated) fumarate hydratase (FH)-deficient renal cell carcinomas (RCC) to the 2016 WHO Classification of Renal Tumours (1) have highlighted an evolving need for the distinction between renal cancer subtypes. Differences in molecular characterisation, clinical phenotypes, and therapeutic responses (1-3) further corroborates this paradigm shift and strongly points towards the development of subtype-specific management (3). SDH and FH-deficient RCCs are rare tumours strongly associated with hereditary neoplastic syndromes and early-onset(2, 4, 5). FH-deficient RCCs are highly aggressive tumours associated with poor patient prognosis(2, 3, 6), whereas SDH-deficient RCCs are phenotypically more variable, but also