The finding that all deceased SDHB-related PGL patients had metastatic PGL suggests that the occurrence of metastatic disease in SDHB-linked PGL syndrome particularly impacts survival, and that metastases may be either more prevalent in SDHB-linked cases, as suggested before [7,10,21,22,23,24], or more aggressive than metastatic diseases associated with other SDHx genes, a finding that is in line with the very poor 5-year survival rate of SDHB-linked metastatic disease reported by Amar et al. [11]. The gene discussed is SDHB; the disease is metastatic neoplasm.