We report a rare case of advanced, metastatic gastrointestinal stromal tumor (GIST) in a young female. Molecular analysis of the tumor revealed wild-type (WT) KIT and platelet derived growth factor receptor alpha (PDGFRA) gene status with no mutations characteristic of adult GIST. Despite this she had clinical benefit and evidence of radiological response to sequential treatment with the tyrosine kinase inhibitors imatinib and sunitinib.
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