TY - JOUR
T1 - Advanced chordoma treated by first-line molecular targeted therapies
T2 - Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF)
AU - Lebellec, Loïc
AU - Chauffert, Bruno
AU - Blay, Jean Yves
AU - Le Cesne, Axel
AU - Chevreau, Christine
AU - Bompas, Emmanuelle
AU - Bertucci, François
AU - Cupissol, Didier
AU - Fabbro, Michel
AU - Saada-Bouzid, Esma
AU - Duffaud, Florence
AU - Feuvret, Loïc
AU - Bonneville-Levard, Alice
AU - Bay, Jacques Olivier
AU - Vauleon, Elodie
AU - Vinceneux, Armelle
AU - Noel, Georges
AU - Penel, Nicolas
AU - Mir, Olivier
N1 - Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/7/1
Y1 - 2017/7/1
N2 - 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.
AB - 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.
KW - Anti-EGFR
KW - Antiangiogenics
KW - Chordoma
KW - Imatinib
KW - Molecular targeted therapy
KW - Prognostic factors
UR - http://www.scopus.com/inward/record.url?scp=85018250678&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2017.03.037
DO - 10.1016/j.ejca.2017.03.037
M3 - Article
C2 - 28478340
AN - SCOPUS:85018250678
SN - 0959-8049
VL - 79
SP - 119
EP - 128
JO - European Journal of Cancer
JF - European Journal of Cancer
ER -