Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: Expert recommendations from the World Sarcoma Network Review


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.; George, S.; Gronchi, A.; Haas, R. L.; Hindi, N.; Hohenberger, P.; Joensuu, H.; Jones, R. L.; Judson, I.; Kang, Y. K.; Kawai, A.; Lazar, A. J.; Le Cesne, A.; Maestro, R.; Maki, R. G.; Martín, J.; Patel, S.; Penault-Llorca, F.; Premanand Raut, C.; Rutkowski, P.; Safwat, A.; Sbaraglia, M.; Schaefer, I. M.; Shen, L.; Serrano, C.; Schöffski, P.; Stacchiotti, S.; Sundby Hall, K.; Tap, W. D.; Thomas, D. M.; Trent, J.; Valverde, C.; van der Graaf, W. T. A.; von Mehren, M.; Wagner, A.; Wardelmann, E.; Naito, Y.; Zalcberg, J.; Blay, J. Y.
Review Title: Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: Expert recommendations from the World Sarcoma Network
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. © 2020 The Author(s)
Keywords: sarcoma; entrectinib; larotrectinib; tropomyosin receptor kinase; neurotrophic tyrosine receptor kinase
Journal Title: Annals of Oncology
Volume: 31
Issue: 11
ISSN: 0923-7534
Publisher: Oxford University Press  
Date Published: 2020-11-01
Start Page: 1506
End Page: 1517
Language: English
DOI: 10.1016/j.annonc.2020.08.2232
PUBMED: 32891793
PROVIDER: scopus
PMCID: PMC7985805
DOI/URL:
Notes: Review -- Export Date: 2 November 2020 -- Source: Scopus
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  1. Cristina R Antonescu
    897 Antonescu
  2. William Douglas Tap
    375 Tap