Combining our series with the previously published reports (3, 7, 8, 11–18), it emerges that GIST can arise at very different age ranges in SDHA-variant carriers, since mean age at diagnosis is 36.0 ± 2.3 years, but up to 22% of patients are being diagnosed at more than 50 years old (Figure 2). Here, SDHA is linked to gastrointestinal stromal tumor.