Interestingly, while the loss of SDHB protein expression detected by IHC is associated with pathogenic SDHA variants, the tumor from the proband had the characteristics of a clear cell carcinoma without additional features typically associated with SDH‐deficient RCC, such as eosinophilic cytoplasm with intracytoplasmic vacuolations and inclusions (Gill, et al., 2014, 2011; Udager & Mehra, 2016). Here, SDHA is linked to clear cell adenocarcinoma.