Identification of NTRK fusions in pediatric mesenchymal tumors

D Pavlick, AB Schrock, D Malicki… - Pediatric blood & …, 2017 - Wiley Online Library
D Pavlick, AB Schrock, D Malicki, PJ Stephens, DJ Kuo, H Ahn, B Turpin, JM Allen…
Pediatric blood & cancer, 2017•Wiley Online Library
Background NTRK fusions are known oncogenic drivers and have recently been effectively
targeted by investigational agents in adults. We sought to assess the frequency of NTRK
fusions in a large series of pediatric and adolescent patients with advanced cancers.
Procedure Genomic profiles from 2,031 advanced cancers from patients less than 21 years
old who were assayed with comprehensive genomic profiling were reviewed to identify
NTRK fusions. Results Total of nine cases (0.44%) harbored NTRK fusions, including novel …
Background
NTRK fusions are known oncogenic drivers and have recently been effectively targeted by investigational agents in adults. We sought to assess the frequency of NTRK fusions in a large series of pediatric and adolescent patients with advanced cancers.
Procedure
Genomic profiles from 2,031 advanced cancers from patients less than 21 years old who were assayed with comprehensive genomic profiling were reviewed to identify NTRK fusions.
Results
Total of nine cases (0.44%) harbored NTRK fusions, including novel partners. Four of these cases were in children less than 2 years old for which infantile fibrosarcoma was considered as a diagnosis, and two harbored the canonical ETV6‐NTRK3. The remaining cases carried other diagnoses, at least one that carried the diagnosis of inflammatory myofibroblastic tumor.
Conclusions
NTRK fusions occur in a subset of young patients with mesenchymal or sarcoma‐like tumors at a low frequency, and are eminently druggable targets via either investigational agents or approved drugs.
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