Gastrointestinal Stromal Tumors PDQ®

In brief

NCI's evidence-based clinical reference for healthcare professionals managing pediatric gastrointestinal stromal tumors (GIST). Covers diagnosis, molecular characteristics, and treatment approaches for these rare mesenchymal neoplasms in children, including targeted therapy options.

• Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms that can occur in pediatric populations with distinct biology from adult GISTs.
• Pediatric GISTs often lack KIT/PDGFRA mutations common in adults, requiring different diagnostic and therapeutic approaches.
• Treatment typically involves surgical resection with consideration of targeted therapy based on molecular profiling.
• Multidisciplinary management is essential given the rarity and complexity of pediatric GIST cases.

Written by the GCMD Library team from the guideline.