title: Efficacy of trabectedin in malignant solitary fibrous tumors: a retrospective analysis from the French Sarcoma Group.
abstract: BACKGROUND
  Advanced malignant solitary fibrous tumors (SFTs) are rare soft-tissue sarcomas with a poor prognosis. Several treatment options have been reported, but with uncertain rates of efficacy. Our aim is to describe the activity of trabectedin in a retrospective, multi-center French series of patients with SFTs.
  
  
  METHODS
  Patients were mainly identified through the French RetrospectYon database and were treated between January 2008 and May 2013. Trabectedin was administered at an initial dose of 1.5 mg/m(2), q3 weeks. The best tumor response was assessed according to the Response Evaluation Criteria In Solid Tumors 1.1. The Kaplan-Meier method was used to estimate median progression-free survival (PFS) and overall survival (OS). The growth-modulation index (GMI) was defined as the ratio between the time to progression with trabectedin (TTPn) and the TTP with the immediately prior line of treatment (TTPn-1).
  
  
  RESULTS
  Eleven patients treated with trabectedin for advanced SFT were identified. Trabectedin had been used as second-line treatment in 8 patients (72.7 %) and as at least third-line therapy in a further 3 (27.3 %). The best RECIST response was a partial response (PR) in one patient (9.1 %) and stable disease (SD) in eight patients (72.7 %). Disease-control rate (DCR = PR + SD) was 81.8 %. After a median follow-up of 29.2 months, the median PFS was 11.6 months (95 % CI = 2.0; 15.2 months) and the median OS was 22.3 months (95 % CI = 9.1 months; not reached). The median GMI was 1.49 (range: 0.11-4.12).
  
  
  CONCLUSIONS
  Trabectedin is a very promising treatment for advanced SFTs. Further investigations are needed.
authors.affiliation: Department of Medical Oncology, Institut Claudius Regaud/Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse Cedex 9, France. jonathan.khalifa@hotmail.fr.
authors.forename: J
authors.initials: J
authors.lastname: Khalifa
authors.affiliation: Department of Statistics, Institut Claudius Regaud / Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse, France. ouali.monia@iuct-oncopole.fr.
authors.forename: M
authors.initials: M
authors.lastname: Ouali
authors.affiliation: Department of Statistics, Institut Claudius Regaud / Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse, France. chaltiel.leonor@iuct-oncopole.fr.
authors.forename: L
authors.initials: L
authors.lastname: Chaltiel
authors.affiliation: Department of Pathology, Institut Claudius Regaud / Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse, France. leguellec.sophie@iuct-oncopole.fr.
authors.forename: S
authors.initials: S
authors.lastname: Le Guellec
authors.affiliation: Department of Medical Oncology, Institut Gustave Roussy, 114 rue Edouard Vaillant, 94805, Villejuif, France. axel.lecesne@igr.fr.
authors.forename: A
authors.initials: A
authors.lastname: Le Cesne
authors.affiliation: Department of Medical Oncology, Centre Léon Bérard, 28 Promenade Léa et Napoléon Bullukian, 69008, Lyon, France. jean-yves.blay@lyon.unicancer.fr.
authors.forename: J-Y
authors.initials: JY
authors.lastname: Blay
authors.affiliation: Department of Medical Oncology, Centre Léon Bérard, 28 Promenade Léa et Napoléon Bullukian, 69008, Lyon, France. philippe.cousin@lyon.unicancer.fr.
authors.forename: P
authors.initials: P
authors.lastname: Cousin
authors.affiliation: Department of Medical Oncology, Jean Minjoz University Hospital, 3 Boulevard Alexandre Fleming, 25030, Besançon, France. chaigneau.loic@orange.fr.
authors.forename: L
authors.initials: L
authors.lastname: Chaigneau
authors.affiliation: Department of Medical Oncology, Institut de Cancérologie de l'Ouest, Site Hospitalier Nord Boulevard Jacques Monod, 44805, Saint-Herblain, France. emmanuelle.bompas@ico.unicancer.fr.
authors.forename: E
authors.initials: E
authors.lastname: Bompas
authors.affiliation: Department of Medical Oncology, Institut Curie, 26 rue d'Ulm, 75248, Paris, France. 8sophie.piperno-neumann@curie.fr.
authors.forename: S
authors.initials: S
authors.lastname: Piperno-Neumann
authors.affiliation: Department of Medical Oncology, Institut Bergonié, 229 cours de l'Argonne, 33000, Bordeaux, France. b.bui@bordeaux.unicancer.fr.
authors.forename: B
authors.initials: B
authors.lastname: Bui-Nguyen
authors.affiliation: Department of Medical Oncology, Centre Alexis Vautrin, 6 Avenue de Bourgogne, 54519, Vandœuvre-lès-Nancy, France. m.rios@nancy.unicancer.fr.
authors.forename: M
authors.initials: M
authors.lastname: Rios
authors.affiliation: Department of Medical Oncology, Institut Claudius Regaud/Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse Cedex 9, France. delord.jean-pierre@iuct-oncopole.fr.
authors.forename: J-P
authors.initials: JP
authors.lastname: Delord
authors.affiliation: Department of Medical Oncology, Centre Oscar Lambret, 3 Rue Frédéric Combemale, 59000, Lille, France. n-penel@o-lambret.fr.
authors.forename: N
authors.initials: N
authors.lastname: Penel
authors.affiliation: Department of Medical Oncology, Institut Claudius Regaud/Institut Universitaire du Cancer de Toulouse - Oncopôle, 1, avenue Irène Joliot-Curie, 31059, Toulouse Cedex 9, France. chevreau.christine@iuct-oncopole.fr.
authors.forename: C
authors.initials: C
authors.lastname: Chevreau
mesh_terms: D000328:Adult; D004149:Dioxoles / Q000008:administration & dosage*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy* / Q000473:pathology; D054364:Solitary Fibrous Tumors / Q000188:drug therapy* / Q000473:pathology; D044005:Tetrahydroisoquinolines / Q000008:administration & dosage*; D000077606:Trabectedin; D016896:Treatment Outcome
chemical_list: D004149:Dioxoles; D044005:Tetrahydroisoquinolines; D000077606:Trabectedin