Targeted therapy with entrectinib in children with NTRK rearranged mesenchymal neoplasms

Non-rhabdomyosarcoma soft-tissue sarcoma (“RMS-like” and “non-RMS-like” tumours- NRSTS) of childhood is a highly heteroge- neous group of tumours. Within the subgroup of undifferentiated sarcomas (UDS), new entities have been described based on mo- lecular markers found through increasingly available genetic analysis in the new 2020 classification of the World Health Organiza- tion (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 pa- tients with soft tissue sarcoma and neurotrophic tyrosine receptor kinase (NTRK) gene rearrangement, focusing on excellent thera- peutic response to NTRK inhibitor targeted therapy with good drug tolerance.

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