VHL and neoplasm: Interestingly, though strong HIF2α staining has been shown in both VHL-mutated and SDH-mutated tumours (Favier et al. 2009; Lopez-Jimenez et al. 2010), PPGL with HIFα mutations demonstrate a different pseudohypoxic transcriptome, suggesting that a further transcriptional driver is involved (Fliedner et al. 2016).