Carney triad (CT) shows the association of paragangliomas (PGLs) with GISTs and pulmonary chondromas in which inactivating mutations of the mitochondrial complex II SDH enzyme subunits SDHB, SDHC and SDHD are found in PGLs, gain-of-function mutations of c-kit (KIT), and platelet-derived growth factor receptor A (PDGFRA) in GISTs81. Here, SDHD is linked to paraganglioma.