Literature DB >> 28478340

Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).

Loïc Lebellec1, Bruno Chauffert2, Jean-Yves Blay3, Axel Le Cesne4, Christine Chevreau5, Emmanuelle Bompas6, François Bertucci7, Didier Cupissol8, Michel Fabbro8, Esma Saada-Bouzid9, Florence Duffaud10, Loïc Feuvret11, Alice Bonneville-Levard3, Jacques-Olivier Bay12, Elodie Vauleon13, Armelle Vinceneux14, Georges Noel15, Nicolas Penel16, Olivier Mir4.   

Abstract

BACKGROUND: To assess the role of first-line Molecular Targeted Therapies (MTTs) in Advanced chordoma (AC) patients.
METHODS: Retrospective study of 80 patients treated between January 2004 and December 2015 at 15 major French Sarcoma or Neurooncology Centres.
RESULTS: The sex ratio M/F was 46/34. The median age was 59 (6-86) years. The primary sites were the sacrum (50, 62.5%), mobile spine (12, 15.0%), and skull base (18, 22.5%). Metastases were present in 28 patients (36.0%). The first line of MTTs consisted of imatinib (62, 77.5%), sorafenib (11, 13.7%), erlotinib (5, 6.3%), sunitinib (1, 1.2%) and temsirolimus (1, 1.2%). The reported responses were: partial response (5, 6.3%), stable disease (58, 72.5%), or progressive disease (10, 12.5%). Symptomatic improvement was seen in 28/66 assessable patients (42.4%) and was associated with an objective response occurrence (p = 0.005), imatinib (p = 0.020) or erlotinib use (p = 0.028). The median progression-free survival (PFS) was 9.4°months (95% CI, [6.8-16.1]). Two independent factors of poor prognosis for PFS were identified: a skull-based primary location (HR = 2.5, p = 0.019), and the interval between diagnosis and MTT of <52months (HR = 2.8, p < 0.001). The median overall survival (OS) was 4.4°years (95% CI, [3.8-5.6]). Four independent factors of poor prognosis for OS were identified: the presence of liver metastases (HR = 13.2, p < 0.001), pain requiring opioids (HR = 2.9, p = 0.012), skull-based primary location (HR = 19.7, p < 0.001), and prior radiotherapy (photon alone) (HR = 2.5, p = 0.024). The PFS and OS did not significantly differ between the MTT.
CONCLUSIONS: The prognostic factors identified require validation in an independent database but are potently useful to guide treatment decisions and design further clinical trials.
Copyright © 2017 Elsevier Ltd. All rights reserved.

Entities:  

Keywords:  Anti-EGFR; Antiangiogenics; Chordoma; Imatinib; Molecular targeted therapy; Prognostic factors

Mesh:

Substances:

Year:  2017        PMID: 28478340     DOI: 10.1016/j.ejca.2017.03.037

Source DB:  PubMed          Journal:  Eur J Cancer        ISSN: 0959-8049            Impact factor:   9.162


  11 in total

1.  Advancement of PI3 Kinase Inhibitor Combination Therapies for PI3K-Aberrant Chordoma.

Authors:  Molly E Heft Neal; Nicole L Michmerhuizen; Kevin J Kovatch; John Henry J Owen; Jingyi Zhai; Hui Jiang; Erin L McKean; Mark E P Prince; J Chad Brenner
Journal:  J Neurol Surg B Skull Base       Date:  2020-10-12

2.  Targeted Therapy for Chordoma: Key Molecular Signaling Pathways and the Role of Multimodal Therapy.

Authors:  Oluwaseun O Akinduro; Paola Suarez-Meade; Diogo Garcia; Desmond A Brown; Rachel Sarabia-Estrada; Steven Attia; Ziya L Gokaslan; Alfredo Quiñones-Hinojosa
Journal:  Target Oncol       Date:  2021-04-24       Impact factor: 4.864

3.  Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma.

Authors:  Maurício Fernando Silva Almeida Ribeiro; Micelange Carvalho de Sousa; Samir Abdallah Hanna; Marcos Vinicius Calfat Maldaun; Ceci Obara Kurimori; Luiz Guilherme Cernaglia Aureliano de Lima; Romulo Loss Mattedi; Rodrigo Ramella Munhoz
Journal:  Case Rep Oncol Med       Date:  2018-04-10

Review 4.  Skull base chordomas review of current treatment paradigms.

Authors:  Dan Yaniv; Ethan Soudry; Yulia Strenov; Marc A Cohen; Aviram Mizrachi
Journal:  World J Otorhinolaryngol Head Neck Surg       Date:  2020-04-18

5.  Defective homologous recombination DNA repair as therapeutic target in advanced chordoma.

Authors:  Stefan Gröschel; Daniel Hübschmann; Francesco Raimondi; Peter Horak; Gregor Warsow; Martina Fröhlich; Barbara Klink; Laura Gieldon; Barbara Hutter; Kortine Kleinheinz; David Bonekamp; Oliver Marschal; Priya Chudasama; Jagoda Mika; Marie Groth; Sebastian Uhrig; Stephen Krämer; Christoph Heining; Christoph E Heilig; Daniela Richter; Eva Reisinger; Katrin Pfütze; Roland Eils; Stephan Wolf; Christof von Kalle; Christian Brandts; Claudia Scholl; Wilko Weichert; Stephan Richter; Sebastian Bauer; Roland Penzel; Evelin Schröck; Albrecht Stenzinger; Richard F Schlenk; Benedikt Brors; Robert B Russell; Hanno Glimm; Matthias Schlesner; Stefan Fröhling
Journal:  Nat Commun       Date:  2019-04-09       Impact factor: 14.919

6.  Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study.

Authors:  Ran Wei; Dylan C Dean; Pichaya Thanindratarn; Francis J Hornicek; Wei Guo; Zhenfeng Duan
Journal:  Front Oncol       Date:  2020-11-17       Impact factor: 6.244

7.  Long Non-Coding RNA MDFIC-7 Promotes Chordoma Progression Through Modulating the miR-525-5p/ARF6 Axis.

Authors:  Kai Zhang; Zixiang Liu; Zhidong Wang; Zhangzhe Zhou; Xiaofeng Shao; Xi Hua; Haiqing Mao; Huilin Yang; Ke Ren; Kangwu Chen
Journal:  Front Oncol       Date:  2021-09-21       Impact factor: 6.244

Review 8.  Translational Windows in Chordoma: A Target Appraisal.

Authors:  Samantha E Hoffman; Sally A Al Abdulmohsen; Saksham Gupta; Blake M Hauser; David M Meredith; Ian F Dunn; Wenya Linda Bi
Journal:  Front Neurol       Date:  2020-07-08       Impact factor: 4.003

Review 9.  Chordoma: a case series and review of the literature.

Authors:  Ozkan Alan; Tugba Akin Telli; Ozlem Ercelep; Eda Tanrikulu Simsek; Tugba Basoglu Tuylu; Aydan Mutis; Rahib Hasanov; Serap Kaya; Nalan Akgül Babacan; Faysal Dane; Perran Fulden Yumuk
Journal:  J Med Case Rep       Date:  2018-08-27

10.  Synergistic drug combinations and machine learning for drug repurposing in chordoma.

Authors:  Edward Anderson; Tammy M Havener; Kimberley M Zorn; Daniel H Foil; Thomas R Lane; Stephen J Capuzzi; Dave Morris; Anthony J Hickey; David H Drewry; Sean Ekins
Journal:  Sci Rep       Date:  2020-07-31       Impact factor: 4.379

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