Autor: |
Emanuela Palmerini, Giorgio Frega, Marco Gambarotti, Tommaso Frisoni, Marilena Cesari, Alberto Bazzocchi, Marco Miceli, Davide Maria Donati, Stefano Fanti, Cristina Nanni, Stefania Benini, Alessandra Longhi, Anna Paioli, Andrea Marrari, Rossella Hakim, Alberto Righi, Toni Ibrahim |
Jazyk: |
angličtina |
Rok vydání: |
2023 |
Předmět: |
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Zdroj: |
Frontiers in Oncology, Vol 13 (2023) |
Druh dokumentu: |
article |
ISSN: |
2234-943X |
DOI: |
10.3389/fonc.2023.1252359 |
Popis: |
Background: Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging. Here, we report the case of a patient affected by EML4-NTRK3 rearranged undifferentiated spindle cell bone sarcoma treated with larotrectinib, and we argue (discuss about) the incidence and clinical presentation of NTRK gene-fusion positive bone sarcomas, the potential use of upfront treatment with NTRK inhibitors in neoadjuvant setting, and the role of a multidisciplinary tumor board. Despite the rarity of these rearrangements in patients with primitive bone sarcomas, the therapy with NTRK inhibitors represents a highly effective strategy to be pursued in selected cases even in neoadjuvant settings. The management of these very rare cancers should always be discussed in a multidisciplinary board of reference centers. |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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