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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

Authors :
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
Source :
Frontiers in Oncology, Vol 13 (2023)
Publication Year :
2023
Publisher :
Frontiers Media S.A., 2023.

Abstract

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.

Details

Language :
English
ISSN :
2234943X
Volume :
13
Database :
Directory of Open Access Journals
Journal :
Frontiers in Oncology
Publication Type :
Academic Journal
Accession number :
edsdoj.65afba1c23ad453fa34d144e8c213b50
Document Type :
article
Full Text :
https://doi.org/10.3389/fonc.2023.1252359