• Rare
    • Fewer than 100 cases are reported
    • Affects predominantly young females
    • No genetic link
    • Generally considered to be a nonhereditary disorder caused by a postzygotic somatic mosaicism epigenetic silencing of the SDHC promoter; this leads to a deficiency of the SDHx enzyme complex
  • Associated with
  • Should be distinguished from the [[Carney-Stratakis dyad]