To date, the medical management of SDH-deficient GISTs is still controversial because of limited data available, both due to the rarity of this molecular subset of GIST and to the lack of SDH deficiency characterization in most studies.110The mortality is almost 15%, although these tumors are unpredictable since metastasis of cancer cells may be initiated after a long time.40, 121. Here, SDHB is linked to cancer.