Titre :

Natural history and treatment efficacy in an ambispective case series of NTRK-rearranged mesenchymal tumors.

Auteur(s) :

Dufresne, A. [Auteur]
Centre Léon Bérard [Lyon]
Pissaloux, D. [Auteur]
Centre Léon Bérard [Lyon]
Ngo, C. [Auteur]
Institut Gustave Roussy [IGR]
Penel, Nicolas [Auteur]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
Le Cesne, A. [Auteur]
Institut Gustave Roussy [IGR]
Macagno, N. [Auteur]
Centre Léon Bérard [Lyon]
Vanacker, H. [Auteur]
Centre Léon Bérard [Lyon]
Hénon, C. [Auteur]
Institut Gustave Roussy [IGR]
Jean-Denis, M. [Auteur]
Centre Léon Bérard [Lyon]
Rughoo, K. [Auteur]
Centre Léon Bérard [Lyon]
Tirode, F. [Auteur]
Centre Léon Bérard [Lyon]
Blay, J. Y. [Auteur]
Centre Léon Bérard [Lyon]
Brahmi, M. [Auteur]
Centre Léon Bérard [Lyon]

Titre de la revue :

European Society for Medical Oncology

Nom court de la revue :

ESMO Open

Numéro :

8

Pagination :

101202

Date de publication :

2023-04-15

ISSN :

2059-7029

Mot(s)-clé(s) en anglais :

TRK inhibitor
NTRK rearrangement
mesenchymal tumor
sarcoma

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Background Apart for infantile fibrosarcoma (IFS), very little is known about NTRK-rearranged mesenchymal tumors (NMTs). The objective of this study is to describe the distribution, characteristics, natural history, and ...
Lire la suite >Background Apart for infantile fibrosarcoma (IFS), very little is known about NTRK-rearranged mesenchymal tumors (NMTs). The objective of this study is to describe the distribution, characteristics, natural history, and prognosis of NMT. Patients and methods This study was carried out as a translational research program, retrospectively from a cohort of 500 soft tissue sarcoma (STS; excluding IFS) and prospectively both in routine practice and from the RNASARC molecular screening program (N = 188; NCT03375437). Results Using RNA-sequencing, NTRK fusion was detected in 16 patient tumors diagnosed as STS: 8 samples of sarcoma with simple genomics (4 NTRK-rearranged spindle cell neoplasm, 3 ALK/ROS wild-type inflammatory myofibroblastic tumor, and 1 quadruple Wild-type gastrointestinal stromal tumor) and 8 samples of sarcomas with complex genomics (dedifferentiated liposarcoma, intimal sarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma, high-grade uterine sarcoma, malignant peripheral nerve sheath tumor). Among the eight patients with simple genomics, four were treated with tyrosine receptor kinase inhibitor (TRKi) at different stages of the disease and all benefited from the treatment, including one complete response. Among the eight other patients, six evolved with metastatic spreading and the median metastatic survival was 21.9 months, as classically reported in these tumor types. Two of them received a first-generation TRKi without objective response. Conclusions Our study confirms low frequency and histotype diversity of NTRK fusion in STS. While the activity of TRKi in simple genomics NMT is confirmed, our clinical data encourage subsequent studies focusing on the biological relevance of NTRK fusions in sarcomas with complex genomics together with the efficacy of TRKi in this population.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
CHU Lille

Collections :

• METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694

Date de dépôt :

2023-11-15T02:09:47Z
2024-04-05T14:01:07Z