Study of Emactuzumab for Tenosynovial Giant Cell Tumor (TGCT)

Inclusion Criteria:

• Age >12 years

• Biopsy-confirmed (standard of care diagnosis history) local or diffuse TGCT wheresurgical resection would be associated with predicted worsening functionallimitations through surgical joint damage, and/or subject has an anticipated highrisk of early recurrence as determined by a multidisciplinary tumour board orequivalent, or any other morbidity associated with the surgery, and/or surgicaltreatment is not expected to improve the clinical outcomes of the subject.

• Measurable disease: longest diameter ≥20 mm.

• Adequate organ and bone marrow function

• If a woman of childbearing potential (WOCBP), must have a negative pregnancy testprior to starting treatment and agree to use a highly effective method ofcontraception

• Participants must have given written consent

Exclusion Criteria:

• If a female, the subject is pregnant or breast feeding.

• Medical conditions, including auto-immune, requiring systemic immunosuppression. Anysystemic treatment for these conditions (eg, glucocorticoids) is not allowed within 4 weeks of Screening and during the study.

• Known metastatic TGCT or other active cancer that requires concurrent or plannedtreatment

• Received systemic therapy for TGCT (investigational or approved) targeting CSF-1 orCSF-1R or any multi-tyrosine kinase inhibitor (eg nilotinib and imatinib) within 3months prior to screening

• Any surgery, chemotherapy or radiotherapy within 3 months of screening

• Unresolved clinically significant toxicity from a previous treatment or any historyof serious liver toxicity.

• Current or chronic history of liver disease.

• Inadequate renal and liver function

• Systemic antiretroviral therapy within 3 months of baseline

• Within 6 months of baseline has experienced: clinically significant myocardialinfarction, severe/unstable angina pectoris, congestive heart failure New York HeartAssociation (NYHA) Class III or IV, or pulmonary disease (NYHA Criteria 1994)including severe thromboembolic event; incompletely healed clinically significantwounds, including bone fractures; pathological fracture or significanthypercalcaemia.