Pearls and Updates on Pediatric Tyrosine Kinase-Altered Tumors.
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Receptor tyrosine kinases (RTKs) are crucial cell surface receptors that activate intracellular signaling pathways in response to external stimuli, regulating cell proliferation and survival. Under normal conditions, RTK activity is tightly regulated. In pediatric tumors, particularly mesenchymal neoplasms, chromosomal rearrangements are the primary mechanism of RTK-driven oncogenesis, involving genes like NTRK1/2/3, ALK, ROS1, RET, EGFR, and PDGFRB. This review highlights recent advances in understanding RTK-driven myofibroblastic tumors, focusing on the molecular characteristics that influence their classification and clinical behavior. Integration of morphologic, immunophenotypic, and molecular information is essential for accurate diagnosis, given the complex overlap among these tumors.