Carney-Stratakis dyad

• Autosomal dominant disorder
• Incomplete penetrance
• Dyad
  • Paragangliomas
  • Gastrointestinal stromal tumor
  • Not associated with pathogenic variants in KIT or the platelet-derived growth factor receptor alpha,
    • Many are attributed to germline pathogenic variants of SDHB, SDHC, or SDHD
• Should be distinguished from the Carney triad
  • GIST
  • Paragangliomas
  • Pulmonary chondroma