Abstract
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome-associated renal cell carcinoma (RCC) is a rare but significant diagnosis due to the need to genetically screen close family members and its aggressive behaviour.1 Patients with HLRCC syndrome carry a germline mutation of the Fumarate Hydratase (FH) gene, resulting in accumulation of fumarate. Other manifestations of this syndrome include uterine leiomyomas.2
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