5 Facts you should know about

Carney-Stratakis syndrome

Characterized by gastrointestinal stromal tumors (GIST) and paragangliomas, often at multiple sites

Unlike the Carney triad, this is considered to be an inherited syndrome

Believed to be caused by SDHD, SDHB, or SDHC germline mutations

GIST are multifocal and the paragangliomas are multicentric

Carney-Stratakis syndrome is caused by SDH defects, suggesting that GIST can be caused by defective mitochondrial oxidation

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