This fact has very important implications supporting the need for genetic counseling, since even though complete pedigree analysis of SDHA mutation carrier families is still lacking and the clinical insights suggest a low penetrance of SDHA mutation in affected families (12, 26), the notion of a lifelong risk for GIST development urges the need for genetic testing and permanent clinical surveillance of SDHA-variant carriers. The gene discussed is SDHA; the disease is gastrointestinal stromal tumor.