Targeted therapy with entrectinibin children with NTRK rearrangedmesenchymal neoplasms

Abstract

Non-rhabdomyosarcoma soft-tissue sarcoma (“RMS-like” and “non-RMS-like” tumours- NRSTS) of childhood is a highly heterogeneous group of tumours. Within the subgroup of undifferentiated sarcomas (UDS), new entities have been described based on molecular markers found through increasingly available genetic analysis in the new 2020 classification of the World Health Organization (WHO). In recent years, the importance of genetic analysis and the detection of molecular tumour markers has been growing due to the possibility of targeted therapy application and the determination of prognosis or disease course. We will present two patients with soft tissue sarcoma and neurotrophic tyrosine receptor kinase (NTRK) gene rearrangement, focusing on excellent therapeutic response to NTRK inhibitor targeted therapy with good drug tolerance.