The risk of metastatic disease for SDHB carriers with PPGL is reported to be about 34-71%; in a study of 64 pediatric PPGL patients with SDHB mutations reported by Jochmanova et al., 70% developed metastases at a median age of 16 years (those patients being diagnosed at a median age of 12 years). The gene discussed is SDHB; the disease is metastatic neoplasm.