- 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
- GIST
- Paragangliomas
- Pulmonary chondroma
- Should be distinguished from the [[Carney-Stratakis dyad]