Gastrointestinal Stromal Tumor….Systemic Therapy Agents and Regimens for Unresectable, Progressive or Metastatic Disease….SDH-deficient GIST: Sunitinib, Regorafenib, Pazopanib, Imatinib/binimetinib (category 2B)

...a total of 62 patients with advanced GIST refractory to imatinib and/or sunitinib were enrolled in this study....For primary mutation analysis, it was found that SDH-deficient patients had longer mPFS (29.1 months) than those with mutations in exon 9 or exon 11 (5.4 and 4.8 months, respectively; P= 0.013)….Regorafenib seems to have better treatment efficacy in GIST patients with SDH deficiency compared to those with primary KIT mutations, and better with secondary mutations in activation loop than those with non-activation loop mutations.