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Case report Gastrointestinal stromal tumors (GISTs) occur exceedingly rare in children and adolescents. Eighty five percent of pediatric GISTs and 15 % of adult GISTs lack oncogenic mutations in KIT and PDGFRA. The results of tyrosine kinase inhibitor therapy in GIST cases with SDH deficiency are limited and controversial. Here, we would like to present a pediatric SDH deficient GIST case treated with surgery and Imatinib Mesylate. We obtained a good response with Imatinib Mesylate.
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