Patient description: The patient is a 60 year old man complaining of frequent headaches, generalized bone pain and difficulty chewing that started 6 years ago and is worsening. Examination shows bilateral swellings around the molars. The swellings have increased since his last examination. Several extraoral lesions are detected in the head and face. The swellings are non-tender and attached to the underlying bone. Further evaluation shows increased uptake of radioactive substance as well as an increase in urinary pyridinoline. His serum alkaline phosphatase is 300 IU/L (the normal range is 44- 147 IU/L). His family history is only significant for hypertension in his mother and DM type 2 in his father. The diagnosis of Paget's Disease of Bone was confirmed and Bisphosphonate will be started as first-line therapy.

Clinical trial eligibility criteria: eligible ages (years): 10.0-999.0,  Ewing Sarcomas Chondrosarcomas Osteosarcomas Chondroma CIC-Rearranged Sarcoma Patients must have histologically confirmed diagnosis of bone sarcoma (osteosarcoma, Ewing sarcoma of bone, chondrosarcoma or chordoma); 2. Patients with confirmed disease progression at study entry; 3. Metastatic disease not amenable to surgical resection or radiation with curative intent; 4. Patients must have measurable disease; 5. Prior treatment : at least one, but no more than two prior chemotherapy regimen for metastatic disease for osteosarcoma, chondrosarcoma and Ewing sarcoma; neo-adjuvant /maintenance therapy are not counted towards this requirement. Chordoma not pretreated or with 1 or 2 prior (combination) chemotherapy regimen or with one or two prior molecularly targeted therapy, but no more than 2 prior lines of treatment (whatever the indication) can be included. At least 4 weeks since last chemotherapy (6 weeks in case of nitrosoureas and mitomycin C), immunotherapy or any other pharmacological treatment and/or radiotherapy; 6. Age ≥10 years for osteosarcomas, Ewing sarcomas and chondrosarcomas (for chordomas, patients must be ≥18 years); 7. Body Surface Area ≥1.30 m²; 8. Life expectancy of greater than 3 months; 9. Eastern Cooperative Oncology Group (ECOG) performance status <2 (Karnofsky ≥60%) for adults patients; 10. Karnofsky scale ≥ 60% for children aged >12 years old / Lansky scale ≥60% for children aged ≤12 years old; 11. Patients must have adequate bone marrow, renal, and hepatic function, as evidenced by the following within 7 days of study treatment initiation: normal organ function as defined below Absolute neutrophil count ≥1.5 Giga/L Platelets ≥100 Giga/L Hemoglobin ≥9 g/dL Serum creatinin ≤1.5 x upper limit of normal (ULN) Glomerular filtration rate (GFR) ≥30 ml/min/1.73 m² according to the modified Diet in Renal Disease (MDRD) abbreviated formula Aspartate transaminase (AST) and alanine transaminase (ALT) ≤2.5 x ULN Bilirubin ≤1.5 X ULN Alkaline phosphatase ≤2.5 x ULN (≤5 x ULN in patient with liver involvement of their cancer). If Alkaline phosphatase >2.5 ULN, hepatic isoenzymes 5-nucleotidase or gamma-glutamyl transferase (GGT) tests must be performed; hepatic isoenzymes 5-nucleotidase must be within the normal range and/or GGT <1.5 x ULN lipase ≤1.5 x ULN  Prior treatment with any VEGFR inhibitor; 2. Soft tissue sarcoma; 3. Other cancer (different histology) within 5 years prior to randomization; 4. Major surgical procedure, open biopsy, significant trauma, within the last 28 days before randomization; 5. Cardiovascular dysfunction Left ventricular ejection fraction (LVEF) <50% Congestive heart failure (New York Heart Association [NYHA]) ≥2 Myocardial infarction <6 months before study Cardiac arrhythmias requiring therapy Uncontrolled hypertension Unstable angina or new-onset angina 6. Arterial or venous thrombotic or embolic events such as cerebrovascular accident (including transient ischemic attacks), deep vein thrombosis, or pulmonary embolism within the last 6 months before randomization; 7. Severe hepatic impairment (Child-Pugh C); 8. Ongoing infection > Grade 2 according to NCI-CTCAE v4.0; 9. Known history of human immunodeficiency virus (HIV) infection; 10. Active hepatitis B or C or chronic hepatitis B or C requiring treatment with antiviral therapy; 11. Difficulties with swallowing study tablets; 12. Prior anticancer therapy, including radiotherapy, endocrine therapy, immunotherapy, chemotherapy (CT) within the last 4 weeks (6 weeks for nitrosoureas and mitomycin C), or other investigational agents ; Concomitant antalgic palliative radiotherapy allowed; 13. Concurrent enrolment in another clinical trial in which investigational therapies are administered; 14. Known hypersensitivity to the active substance or to any of the excipients; 15. Pregnant women, women who are likely to become pregnant or are breast-feeding; 16. For adult patients, individual deprived of liberty or placed under the authority of a tutor; 17. Patients with any psychological, familial, sociological or geographical condition potentially hampering compliance with the study protocol and follow-up schedule; those conditions should be discussed with the patient before registration in the trial; 18. Patients with history of non compliance to medical regimens or unwilling or unable to comply with the protocol; 19. Interstitial lung disease with ongoing signs and symptoms at the time of informed consent; 20. Non-healing wound, non-healing ulcer, or non-healing bone fracture; 21. Patients with evidence or history of any bleeding diathesis, irrespective of severity; 22. Any hemorrhage or bleeding event ≥ CTCAE Grade 3 within 4 weeks prior to the start of study medication; 23. Use of biological response modifiers, such as granulocyte colony stimulating factor (G-CSF), within 3 weeks of study entry