Axitinib and Pembrolizumab in Subjects With Advanced Alveolar Soft Part Sarcoma and Other Soft Tissue Sarcomas

Inclusion Criteria

• Patients must have histologically confirmed sarcoma with pathology review required for any outside samples.
• The following histologies may be enrolled without prior treatment:
• alveolar soft part sarcoma,
• clear cell sarcoma,
• epithelioid hemangioendothelioma, and
• chordoma.
• The following histologies may be enrolled only if refractory to anthracycline-based chemotherapy or if the patient refuses to undergo standard of care treatment:
• synovial sarcoma,
• rhabdomyosarcoma,
• malignant peripheral nerve sheath tumors,
• dedifferentiated, pleomorphic or myxoid/round cell liposarcoma,
• leiomyosarcoma,
• malignant phylloides tumor,
• high grade undifferentiated pleomorphic sarcomas (HGUPS/MFH),
• angiosarcoma,
• spindle cell sarcoma, not otherwise specified (NOS)
• malignant myoepithelioma.
• The following histologies may be enrolled only if refractory to at least one line of chemotherapy or if the patient refuses to undergo standard of care treatment:
• solitary fibrous tumor/hemangiopericytoma.
• The following histologies may be enrolled only if refractory to at least first-line targeted therapy or if the patient refuses to undergo standard of care treatment:
• gastrointestinal stromal tumors,
• extraskeletal myxoid chondrosarcoma,
• PEComa.
• Primary tumors of bone including Ewing's sarcoma, osteosarcoma, and dedifferentiated chondrosarcoma may only be enrolled if there are measurable target lesions occurring in soft tissue and they are refractory to standard of care anthracycline-based chemotherapy.
• Any other histology or standard of care therapy not specifically addressed will be reviewed by the principal investigator and pathologist for final determination of eligibility.
• Measurable disease as defined by RECIST v1.1.
• Radiographic progression as defined by RECIST v1.1, based on comparison between two radiographic studies no greater than 6 months apart.
• Inability to undergo complete resection of the disease by surgery.
• Adequate organ function as defined:
• Hematological
• Absolute neutrophil count (ANC) ≥1, 000 / microliter (mcL)
• Platelets ≥75,000 / mcL
• Hemoglobin ≥8 g/dL without transfusion or erythropoietin (EPO) dependency (within 7 days of assessment)
• Renal
• Serum creatinine ≤1.5 X upper limit of normal (ULN) OR Measured or calculated creatinine clearance ≥ 60 mL/min for subject with creatinine levels > 1.5 X institutional ULN. (GFR can also be used in place of creatinine or CrCl). Creatinine clearance should be calculated per institutional standard.
• Hepatic
• Serum total bilirubin ≤ 1.5 X ULN OR Direct bilirubin ≤ ULN for subjects with total bilirubin levels > 1.5 ULN.
• Aspartate Aminotransferase (AST/SGOT) and Alanine Transaminase (ALT/SGPT) ≤ 2.5 X ULN OR ≤ 5 X ULN for subjects with liver metastases.
• Albumin >2.5 mg/dL
• Coagulation
• International Normalized Ratio (INR) or Prothrombin Time (PT) ≤1.5 X ULN unless subject is receiving anticoagulant therapy as long as PT or PTT is within therapeutic range of intended use of anticoagulants.
• Activated Partial Thromboplastin Time (aPTT) ≤1.5 X ULN unless subject is receiving anticoagulant therapy as long as PT or PTT is within therapeutic range of intended use of anticoagulants.
• Age ≥ 16 years.
• Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1.
• Patients must consent and be willing to undergo three core needle biopsies at baseline, prior to starting Cycle 3, and at off-study. At least one tumor site must be amenable to biopsy in the judgment of the interventional radiologist.
• Female subject of childbearing potential should have a negative urine or serum pregnancy within 72 hours prior to receiving the first dose of study medication. If the urine test is positive or cannot be confirmed as negative, a serum pregnancy test will be required.
• Females of child bearing potential that are sexually active must agree to either practice 2 medically accepted highly effectiv