After the initial report of bi-allelic SDHA inactivation in a PGL case (Burnichon et al., 2010), two reports from the same group identified inactivating SDHA mutations in four unselected WT GIST cases, first by whole-transcriptome next-generation sequencing in two cases (Pantaleo et al., 2011a), and subsequently in two additional cases by conventional Sanger sequencing (Pantaleo et al., 2011c). Here, SDHA is linked to gastrointestinal stromal tumor.