Clinical Heterogeneity and Long‐Term Outcomes of Pediatric Gastrointestinal Stromal Tumors: A Series of Four Patients Highlighting SDH‐Deficient and SDH‐Intact Subtypes

ABSTRACT

Pediatric gastrointestinal stromal tumors (GISTs) are rare, often lacking KIT or platelet‐derived growth factor receptor‐alpha ( PDGFRA ) mutations and driven by succinate dehydrogenase (SDH) deficiency. We report four pediatric patients (three females and one male) managed over a 19‐year period. Two female patients had SDH‐deficient tumors; one suffered metastatic relapse, illustrating the recurrent clinical course of this subtype. Of the two remaining cases, one SDH‐intact female remains in remission 8 years post‐diagnosis. The male patient demonstrated imatinib resistance but responded to sunitinib, facilitating gastrectomy. Despite a local recurrence managed by secondary wedge resection, he remains in remission. Comprehensive molecular profiling is essential for navigating GIST's clinical heterogeneity.