It may be the index tumour in PGL1 or PGL4, presenting at mean age of 37 years (range 14–76), and indeed in many cases it is the sole tumour: two-thirds of patients with SDH-deficient renal cancers had not developed metachronous PPGLs or GISTs by time of report, although many of these patients were still at relatively young age (Gill et al. 2014a, Paik et al. 2014). This evidence concerns the gene SDHB and renal carcinoma.