Clinical Heterogeneity and Long-Term Outcomes of Pediatric Gastrointestinal Stromal Tumors: A Series of Four Patients Highlighting SDH-Deficient and SDH-Intact Subtypes.
Hala Elfarraj, Maha Barbar, Iyad Sultan et al.
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Case in brief
This report details four pediatric patients with gastrointestinal stromal tumors (GISTs) over 19 years. Two patients had SDH-deficient tumors, with one experiencing metastatic relapse. The SDH-intact female patient remains in remission 8 years post-diagnosis. The male patient showed resistance to imatinib but responded to sunitinib, allowing for successful gastrectomy and subsequent management of local recurrence, leading to ongoing remission.
Diagnostic / clinical pearl
Molecular profiling is crucial in pediatric GISTs to understand the clinical heterogeneity and guide management strategies.