Study of Nilotinib Efficacy in Pigmented Villo-Nodular Synovitis/ Tenosynovial Giant Cell Tumour (PVNS/TGCT)

Inclusion Criteria: * Age \> 18 years * Histologically confirmed diagnosis of inoperable progressive or relapsing PVNS/TGCT OR resectable tumour requesting mutilating surgery * Demonstrated progressive disease in the last 12 months * At least one measurable site of disease on MRI/CT scan according to RECIST criteria (RECIST version 1.1) based on investigator's assessment * WHO Performance status of 0, 1 or 2 * Adequate organ, electrolyte and marrow function, defined as the following: serum bilirubin \> or =1.5 x ULN, ALT and AST \< or = 2.5 x ULN, serum creatinine \< or = 1.5 x ULN or creatinine clearance \> or = 50 mL/min, absolute neutrophil count (ANC) \> or = 1.5x109/L, platelets \> or = 100x109/L, serum lipase \< or =1.5 x ULN, magnesium ≥ lower limit of normal (LLN) and potassium ≥ LLN * Prior adequate physical examination including weight, height, ECOG PS and vital signs (systolic and diastolic blood pressure, heart rate after at least 5 minutes in supine position) * Signed written informed consent form * Covered by a medical insurance (in countries where applicable) Exclusion Criteria: * Pregnant or lactating female or female of child-bearing potential not employing adequate contraception during the study and for up to three months following termination of the study * Known hypersensitivity to nilotinib or to any of the excipients, galactose intolerance, lactase deficiency or glucose-galactose malabsorption prior to enrollment * Acute or chronic uncontrolled liver …