Molecular Characterization of Inflammatory Tumors Facilitates Initiation of Effective Therapy

We present a girl aged 11 years with aTFG-ROS1 fusion –positive tumor of the lung that was initially diagnosed as an immunoglobin G4–related inflammatory pseudotumor. She underwent complete left-sided pneumonectomy and later recurred with widely metastatic disease. We then report the case of a boy aged 9 years with widely metastaticTFG-ROS1 fusion –positive IMT with rapid molecular diagnosis. In both children, there was an excellent response to oral targeted therapy. These cases reveal that rapid molecular testing of inflammatory tumors is not only important for diagnosis but also reveals therapeutic opportunities. Targeted inhibitors produ ce significant radiologic responses, enabling potentially curative treatment approaches for metastatic ROS1 fusion IMT with previously limited treatment options. Primary care pediatricians and pediatric subspecialists have a crucial role in the early consultation of a pediatric oncology center exper ienced in molecular diagnostics to facilitate a comprehensive evaluation for children with inflammatory tumors.
Source: PEDIATRICS - Category: Pediatrics Source Type: research