Although it is outside the scope of this review, some preclinical results suggest that HIF2A inhibitors may have applications in other types of RCC in which the abnormal stabilization of HIF2A occurs due to mechanisms that are not caused by VHL loss, for example, in patients with SDH and FH mutations, and even more widely in tumors where the abnormal stabilization of HIF2A occurs as a consequence of prolonged hypoxia. Here, FH is linked to renal cell adenocarcinoma.