Study on TSR-042 in Advanced Clear Cell Sarcoma

Inclusion Criteria:

1. Written informed consent
2. Histological centrally confirmed diagnosis of clear cell sarcoma
3. Availability of archived tumor tissue block, or 15 slides.
4. Locally advanced disease
5. Measurable disease based on RECIST 1.1
6. Patient can be naive or previously treated with 1 or 2 systemic regimens given for recurrent and/or metastatic disease
7. Eastern Cooperative Oncology Group (ECOG) Performance Status ≤ 2
8. Adequate bone marrow function
9. Adequate organ function
10. Cardiac ejection fraction ≥50%
11. At least 18 years of age on day of signing informed consent.
12. Non-pregnant female patients
13. Non-ot breastfeed during the study for 90 days after the last dose of study treatment.
14. Male participant agrees to use an adequate method of contraception
15. No history of arterial and/or venous thromboembolic event within the previous 12 months.
16. Participant receiving corticosteroids may continue as long as their dose is stable for least 4 weeks prior to initiating protocol therapy.
17. Patients who are willing and able to comply with scheduled visits, treatment plan, laboratory tests, and other study procedures.

Exclusion Criteria:

1. Participant must not be simultaneously enrolled in any interventional clinical trial
2. Previous treatment with any non-investigational agents within 14 days of first day of study drug dosing.
3. Must not have received investigational therapy ≤ 4 weeks, or within a time interval less than at least 5 half-lives of the investigational agent, whichever is shorter, prior initiating protocol therapy
4. Other primary malignancy with \<5 years clinically assessed disease-free interval, except basal cell skin cancer, cervical carcinoma in situ, or other neoplasms judged to entail a low risk of relapse
5. Previous treatment with radiation therapy within 14 days of first day of study drug dosing, or patients who have not recovered from adverse events due to agents administered more than 4 weeks earlier
6. Has known active central nervous system (CNS) metastases, leptomeningeal metastases, and/or carcinomatous meningitis. Subjects with previously treated brain metastases may participate provided they are stable, have no evidence of new or enlarging brain metastases, and are not using steroids for at least 7 days prior to trial treatment. This exception does not include carcinomatous meningitis which is excluded regardless of clinical stability
7. Has active, non-infectious pneumonitis
8. Has an active infection requiring systemic therapy
9. Has received prior therapy with an anti-PD-1, anti-PD-L1, or anti-PD-L2 agents
10. Has received a live vaccine within 30 days of planned start of study therapy
11. Major surgery within 3 weeks prior to study entry
12. Any one of the following currently or in the previous 6 months:

Myocardial infarction, congenital long QT syndrome, Torsades de Pointes, arrhythmias right bundle branch block and left anterior hemiblock unstable angina coronary/peripheral artery bypass graft, symptomatic congestive heart failure New York Heart Association Class III or IV, cerebrovascular accident, or transient ischemic attack symptomatic pulmonary embolism. Ongoing cardiac dysrhythmias of Grade \>=3, atrial fibrillation of any grade,or QTcF interval \>470 msec 14. Severe and/or uncontrolled medical disease 15. Patient experienced ≥ Grade 3 immune-related AE with prior immunotherapy 16. Participant has a diagnosis of immunodeficiency or has receiving systemic steroid therapy or any other form of immunosuppressive therapy within 7 days prior to initiating protocol therapy 17. Any known active hepatitis B or hepatitis C 18. Any known history of human immunodeficiency virus 19. Subjects who have current active hepatic or biliary disease (with exception of patients with Gilbert's syndrome, asymptomatic gallstones, liver metastases or stable chronic liver disease per investigator assessment) 20. Expected non-compliance to medical regimens 21. Known history of interstitial lung disease 22. Active autoimmune disease that has required systemic treatment in the past 2 years 23. Known severe hypersensitivity reactions to monoclonal antibodies, any history of anaphylaxis, or uncontrolled asthma