Publication:
Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: expert recommendations from the World Sarcoma Network.

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Date

2020-09-03

Authors

Demetri, G D
Antonescu, C R
Bjerkehagen, B
Bovée, J V M G
Boye, K
Chacón, M
Dei Tos, A P
Desai, J
Fletcher, J A
Gelderblom, H

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Abstract

Sarcomas are a heterogeneous group of malignancies with mesenchymal lineage differentiation. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as tissue-agnostic oncogenic drivers has led to new personalized therapies for a subset of patients with sarcoma in the form of tropomyosin receptor kinase (TRK) inhibitors. NTRK gene rearrangements and fusion transcripts can be detected with different molecular pathology techniques, while TRK protein expression can be demonstrated with immunohistochemistry. The rarity and diagnostic complexity of NTRK gene fusions raise a number of questions and challenges for clinicians. To address these challenges, the World Sarcoma Network convened two meetings of expert adult oncologists and pathologists and subsequently developed this article to provide practical guidance on the management of patients with sarcoma harboring NTRK gene fusions. We propose a diagnostic strategy that considers disease stage and histologic and molecular subtypes to facilitate routine testing for TRK expression and subsequent testing for NTRK gene fusions.

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Adult
Gene Fusion
Humans
Oncogene Proteins, Fusion
Protein Kinase Inhibitors
Receptor, trkA
Sarcoma
Tropomyosin

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Keywords

entrectinib, larotrectinib, neurotrophic tyrosine receptor kinase, sarcoma, tropomyosin receptor kinase

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