On the contrary, SDHBIHC-, but SDHAIHC+ subgroup include 1) cases of syndromic GIST arising from the Carney-Stratakis Syndrome (CSS), that are characterized by SDHB, SDHC or SDHD inactivating mutations (SDHBmut, SDHCmut, or SDHDmut); and 2) cases of Carney Triad (CT), that lack SDHx-mutations [6, 22–24]. Here, SDHB is linked to gastrointestinal stromal tumor.