Paris- RP Reims Rennes Tours Marseille Montpellier Lyon Nice Lille Strasbourg Bordeaux Dijon Besançon Nantes Toulouse Poitiers Angers Nancy Caen Rouen Clermont-Ferrand GSF: Groupe Sarcome Français GETO: Groupe d’Etudes des Tumeurs Osseuses Villejuif N = 36 institutions Sarcoma Database: 3300 pts Frozen tissue bank: 1300 sarcomas

Sarcome 01 Euro-Ewing 99 PI : Dr O. Oberlin – Villejuif EORTCSFCEGPOHUKCCSGTotalN Molecular analysis on Iry: 448 patients (79 %) BM: 326 patients (57%) 30 sept 06 Suspension of R1 randomization

Sarcome 02 PALSAR II PI : Dr N B BUI – Bordeaux Main end-point: overall survival Statistical hypothesis: 3-yr OS: 40% vs 15% (N® per arm: 50) October 06: N = 207 patientsN® = 65/100 patients

Sarcome 03 Osteosarcoma PI : A Le Cesne – Villejuif Localized operable osteosarcoma API-AI regimen 4 CT courses Main end-point: histological response rate Good histological response: 37%ASCO 06 2-yr overall survival:86% 2-yr PFS74% Follow-up Follow-up

Sarcome 04 EWING PI : O Oberlin – Villejuif D1 D8 D15 D21 D28/1 D8 D15 D21 D28 CDDPVP-16 Main end-point: Objective response rate Statistical hypothesis: Simon: at least 6/29 OR October 2006 = 44/45 ASCO 07

Sarcome 05 Desminib PI : JY Blay – Lyon Progressive AF/Desmoid T Glivec®: 400 mg/d 800 mg/d if PD Duration: one year Main end-point: 3-month PFS (at least 7 patients not PD) At 3 month: 1 CR, 1 PR, 31 SD, 5 PD ASCO 06 6-m PFS 73%; 1-yr PFS 69% Translational research ongoing imatinibRandomNSAID

Sarcome 06: Angiotax PI : N Penel – Lille Angiosarcoma 2nd/3rd CT line Weekly taxol: 80 mg/w 3 weeks on, 1 off Main end-point: Objective response rate Statistical hypothesis: Simon two step: patients at least 6/25 OR Secondary end-point: PFS, OS, tolerance Translational research ASCO 2007

Sarcome 07 Taxogem PI : F. Duffaud – Marseille P. Pautier - Villejuif ® phase II Leiomyosarcoma Uterine vs other 2nd CT line N = Main end-point: OR Secondary end-point: PFS, OS, tolerance, QOL N = 13/80

PI (FSG) : A Le Cesne – Villejuif Sarcome 08:Adjuvant GIST EORTC October 2006 N = 418 Participating French centers : 42 Mutational analysis: 70%

PI : F Duffaud – Curie / L Brugieres - Villejuif Intergroup SFCE/GSF/GETO Adult/children OS – phase III Zoledronate N = yr EFS > 13% in exp arm Translationalresearch Sarcome 09 OS 2006

PI: P Pautier – Villejuif Uterine leiomyoS, carcinoS, high grade endometrial S N = 52 / yr EFS > 20% in experimental arm Sarc-Gyn 1 Adjuvant CT Arm A 4 x API RTE 45 Gy + Brachytherapy 15 Gy A (50), I ( 6), C (75) Surgery < 6 w R RTE 45 Gy + Brachytherapy 15 Gy - Arm B

BFR 14 trial - Advanced GIST (June 2002) Stop Stop Imatinib Non PD R Imatinib Non PD R Imatinib Imatinib PI : J.Y Blay – Lyon A. Le Cesne - Villejuif N patients included in October 2005:271 1) N randomized (interrupted) pts at 1 year: 58 (ASCO 04,05) Stop imatinib arm:32 imatinib arm:26 2) N randomized (ongoing) pts at 3 years: 32 (ASCO 07)

AB1010 first line - Advanced GIST Day 1 Preclinical efficacy profile > to IM IC50 < µM vs IC50 = 0.02 µM Phase II AB1010: 7.5 mg/kg 4 centers N = 25 (06/05 to 11/06) ASCO 2007 M6 Day 1 M1 PI : A. Le Cesne - Villejuif