With Reed’s syndrome, the neoplastic process is triggered through biallelic FH gene inactivation, fumarate accumulation in the cells, changes in cell metabolism, the stabilisation of hypoxia-inducible factors (HIFs), the activation of carcinogenic transcription factor NRF2 (nuclear factor erythroid 2 related factor 2) and changes to the expression profile [9,10]. Here, NFE2L2 is linked to hereditary leiomyomatosis and renal cell cancer.