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Pediatr Blood Cancer. 2017 Aug;64(8). doi: 10.1002/pbc.26433. Epub 2017 Jan 18.

Identification of NTRK fusions in pediatric mesenchymal tumors.

Author information

1
Foundation Medicine, Inc., Cambridge, Massachusetts.
2
Department of Pathology, University of California San Diego School of Medicine, La Jolla, California.
3
Department of Pathology, Rady Children's Hospital San Diego, San Diego, California.
4
Division of Pediatric Hematology/Oncology, Rady Children's Hospital San Diego, San Diego, California.
5
Division of Pediatric Hematology/Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
6
Department of Pathology, Nemours Children's Hospital, Orlando, Florida.
7
Department of Pathology, Albany Medical Center, Albany, New York.

Abstract

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.

KEYWORDS:

IFS; IMT; NTRK1-3; kinase fusion; mesoblastic nephroma; sarcoma

PMID:
28097808
DOI:
10.1002/pbc.26433
[Indexed for MEDLINE]

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