Abstract
Abstract BACKGROUND Neurotrophic tyrosine receptor kinases (NTRK 1-3)fusions have been identified in a range of pediatric cancer. Among children with cancer NTRK fusions can be detected in less than 1% of cases. Low grade gliomas (LGG) are the most pediatric brain tumors and the frequency of NTRK fusions reported is between 0.4 -4.3%. NTRK2 fusions are the most commonly reported in pediatric brain tumors. METHOD We describe the characteristics of patients analysed. Due to limited access to Next Generation Sequencing (NGS) only patients with recurrent, progressive, metastatic or midline location LGG were tested during 2021-2022. Positivity to Pan-Trk immunohistochemistry was needed to proceed to confirmation with NGS. 10 patients with LGG were included. Median age of patients at baseline was 4.7 (range,1-12) years. 10/10 showed positivity to Pan-Trk stain and NTRK fusions were detected in 6/10 patients, 3/6 had NTRK1 fusions, 2/6 NTRK2 fusion and 1 with NTRK3 fusion.. All 4 patients without NTRK fusions are off therapy (more than 1 year survellaince) and 2/4 were treated with surgery only. All patients with NTRK fusions are still on treatment due to progressive disease, 4/6 patients have required 2 or more surgeries and 5/6 patients have needed 2 or more chemotherapy regimens. CONCLUSION This small cohort of patients represents a highly selected population with LGG and the frequency of NTRK fusions resulted significantly higher than expected (6 out of 10 patients tested). This finding could reflect a clinical or prognostic significance, it will be important to analyze a larger cohort of patients that share these same clinical characteristics to determine its real value as a prognostic marker in these heavily treated patients. There is well known molecular alterations that predict outcome in patients with LGG. NTRK targeted therapy already exists with proven efficacy although access may limits its use.
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