1999), which is associated with an increased risk of Wilms tumour, hepatoblastoma, neuroblastoma, rhabdomyosarcoma and other embryonal cancers. Interestingly, with inherited SDHD GPVs, where (almost) only paternal transmission of the GPV causes paraganglioma susceptibility in the offspring, SDHD is apparently not an imprinted gene (Burnichon et al. 2017). The gene discussed is SDHD; the disease is Wilms tumor.