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NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report

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 rearran...

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Autores principales: Palmerini, Emanuela, Frega, Giorgio, Gambarotti, Marco, Frisoni, Tommaso, Cesari, Marilena, Bazzocchi, Alberto, Miceli, Marco, Donati, Davide Maria, Fanti, Stefano, Nanni, Cristina, Benini, Stefania, Longhi, Alessandra, Paioli, Anna, Marrari, Andrea, Hakim, Rossella, Righi, Alberto, Ibrahim, Toni
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10591071/
https://www.ncbi.nlm.nih.gov/pubmed/37876963
http://dx.doi.org/10.3389/fonc.2023.1252359
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author Palmerini, Emanuela
Frega, Giorgio
Gambarotti, Marco
Frisoni, Tommaso
Cesari, Marilena
Bazzocchi, Alberto
Miceli, Marco
Donati, Davide Maria
Fanti, Stefano
Nanni, Cristina
Benini, Stefania
Longhi, Alessandra
Paioli, Anna
Marrari, Andrea
Hakim, Rossella
Righi, Alberto
Ibrahim, Toni
author_facet Palmerini, Emanuela
Frega, Giorgio
Gambarotti, Marco
Frisoni, Tommaso
Cesari, Marilena
Bazzocchi, Alberto
Miceli, Marco
Donati, Davide Maria
Fanti, Stefano
Nanni, Cristina
Benini, Stefania
Longhi, Alessandra
Paioli, Anna
Marrari, Andrea
Hakim, Rossella
Righi, Alberto
Ibrahim, Toni
author_sort Palmerini, Emanuela
collection PubMed
description 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.
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spelling pubmed-105910712023-10-24 NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report Palmerini, Emanuela Frega, Giorgio Gambarotti, Marco Frisoni, Tommaso Cesari, Marilena Bazzocchi, Alberto Miceli, Marco Donati, Davide Maria Fanti, Stefano Nanni, Cristina Benini, Stefania Longhi, Alessandra Paioli, Anna Marrari, Andrea Hakim, Rossella Righi, Alberto Ibrahim, Toni Front Oncol Oncology 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. Frontiers Media S.A. 2023-10-09 /pmc/articles/PMC10591071/ /pubmed/37876963 http://dx.doi.org/10.3389/fonc.2023.1252359 Text en Copyright © 2023 Palmerini, Frega, Gambarotti, Frisoni, Cesari, Bazzocchi, Miceli, Donati, Fanti, Nanni, Benini, Longhi, Paioli, Marrari, Hakim, Righi and Ibrahim https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Palmerini, Emanuela
Frega, Giorgio
Gambarotti, Marco
Frisoni, Tommaso
Cesari, Marilena
Bazzocchi, Alberto
Miceli, Marco
Donati, Davide Maria
Fanti, Stefano
Nanni, Cristina
Benini, Stefania
Longhi, Alessandra
Paioli, Anna
Marrari, Andrea
Hakim, Rossella
Righi, Alberto
Ibrahim, Toni
NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title_full NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title_fullStr NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title_full_unstemmed NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title_short NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
title_sort ntrk rearranged sarcoma of the bone. role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10591071/
https://www.ncbi.nlm.nih.gov/pubmed/37876963
http://dx.doi.org/10.3389/fonc.2023.1252359
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