Toripalimab Combined With CAV/IE Regimen

Inclusion Criteria: 1. Patients voluntarily participated in the study and signed informed consent; For all advanced or non resectable bone and soft tissue sarcomas confirmed by pathology, the standard treatment failed or there was no standard treatment. They were mainly synovial sarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma, liposarcoma, fibrosarcoma, clear cell sarcoma, angiosarcoma, epithelioid sarcoma, malignant peripheral nerve sheath tumor, undiffertiated sarcoma, bone sarcoma, chondrosarcoma, Ewing's sarcoma, rhabdomyosarcoma, dermatofibrosarcoma protuberans, myofibroblastic sarcoma, malignant solitary fibroma, postradiation sarcoma,etc. But the pathological subtypes without standard treatment can be treated as first-line treatment, including but not limited to radiation-induced sarcoma, dedifferentiated / pleomorphic liposarcoma, clear cell sarcoma, etc., except for the following types: well differentiated liposarcoma, malignant mesothelioma, gastrointestinal stromal tumor, etc; 2. Advanced patients with unresectable lesions or lymph nodes or distant metastasis assessed by imaging; 3. In the past three months, there was at least one measurable target lesion according to RECIST version 1.1 standard, and it can be accurately measured by magnetic resonance imaging (MRI) or computer tomography (CT) in at least one direction (the maximum diameter needs to be recorded), with conventional CT ≥ 20 mm or spiral CT ≥ 10 mm. 4. They were 14-70 years old; ECOG PS score: 0-1; the expected survival time was more than 3 months; 5. Within 7 days before treatment, the main organ functions met the following criteria: (1) Blood routine examination standard (without blood transfusion within 14 days) ① Hemoglobin (HB) ≥ 90g / L; ② The absolute value of neutrophil (ANC) ≥ 1.5 × 109 / L; * Platelet (PLT) ≥ 80 × 109 / L. (2) Biochemical examination should meet the following standards: * Total bilirubin (TBIL) ≤ 1.5 times the upper limit of normal value (ULN); * Alanine aminotransferase (ALT) and aspartate aminotransferase AST ≤ 2.5uln; ALT and AST ≤ 5uln with liver metastasis * Serum creatinine (CR) ≤ 1.5uln or creatinine clearance rate (CCR) ≥ 60ml / min; (3) Doppler ultrasound evaluation: left ventricular ejection fraction (LVEF) ≥ the lower limit of normal value (50%). 6)Women of childbearing age should agree that contraceptive measures (such as intrauterine device, contraceptive or condom) must be used during the study period and within 6 months after the end of the study; serum or urine pregnancy test negative within 7 days before study enrollment, and must be non lactating patients; men should agree that contraceptive measures must be used during the study period and within 6 months after the end of the study period. Exclusion Criteria: 1\) Patients who had previously received anti-PD-1 / PD-L1 antibody therapy. 2\) Other malignancies occurred or were present within 5 years, except for cervical carcinoma in situ, non melanoma skin cancer and superficial bladder tumor \[ta (non invasive tumor), tis (carcinoma in situ) and T1 (tumor infiltrating basement membrane)\]; 3\) Systemic anti-tumor therapy including cytotoxic therapy, signal transduction inhibitors, immunotherapy (or mitomycin C within 6 weeks prior to the trial drug treatment) was planned within 4 weeks before enrollment or during the study period. Over extended field radiotherapy was performed within 4 weeks before admission or limited field radiotherapy was performed within 2 weeks before grouping; 4\) With pleural effusion or ascites, it causes respiratory syndrome (≥ CTC AE grade 2 dyspnea \[grade 2 dyspnea refers to shortness of breath with a small amount of activity; it affects instrumental activities of daily living\]); 5\) Any unrelieved toxic reaction higher than CTC AE (4.01) grade 1 or above caused by previous treatment, excluding alopecia; 6\) Patients with any severe and / or uncontrolled disease, including: 1. Patients with poor blood pressure control (SBP ≥ 150 mmHg, DBP ≥ 100 mmHg); 2. Patients with myocardial ischemia or myocardial infarction of grade I or above, arrhythmia (including QTc ≥ 480ms) and congestive heart failure (NYHA) grade ≥ 2; 3. Active or uncontrolled severe infection (≥ CTC AE Level 2 infection); 4. Chronic liver disease, decompensated liver disease or decompensated hepatitis; 5. Renal failure needs hemodialysis or peritoneal dialysis; 6. Poor control of diabetes mellitus (FBG \> 10mmol / L); 7. Urine routine examination showed that urine protein was ≥ + +, and 24-hour urine protein was more than 1.0 G; 8. Patients with epilepsy and need treatment; 7\) Major surgical treatment, open biopsy or obvious traumatic injury were performed within 28 days before admission; 8\) Patients with any physical signs or history of bleeding regardless of severity; patients with any bleeding or bleeding events ≥ CTCAE 3 within 4 weeks before enrollment had unhealed wounds, ulcers or fractures; 9\) Patients who had AVT events within 6 months, such as cerebrovascular accident (including transient ischemic attack), deep vein thrombosis and pulmonary embolism; 10\) There were active ulcer, intestinal perforation and intestinal obstruction; 11\) Subjects with clinical symptoms of central nervous system metastasis (such as brain edema, need for hormone intervention, or brain metastasis progression); patients who have previously received treatment for brain or meningeal metastasis, such as clinical stability (MRI) for at least 2 months, and who have stopped systemic hormone therapy (dose \> 10mg / day, prednisone or other effective hormones) for more than 2 weeks can be included; 12\) The subjects were using immunosuppressive agents, or systemic or absorbable local hormone therapy to achieve the purpose of immunosuppression (dosage \> 10mg / D, prednisone or other effective hormones), and continued to use them within 2 weeks before enrollment; 13\) Subjects with any active autoimmune disease or history of autoimmune diseases (e.g., but not limited to: interstitial pneumonia, uveitis, enteritis, hepatitis, hypophysitis, vasculitis, nephritis, hyperthyroidism, hypothyroidism; if the subject has vitiligo or asthma has been completely relieved in childhood, it is not necessary to be an adult Any intervention can be included; asthma patients who need bronchodilator for medical intervention cannot be included); 14\) The subjects had active tuberculosis; 15\) According to the judgment of the researcher, the subjects are not suitable to be enrolled or there are other factors that may lead to termination of the study, such as other serious diseases (including mental diseases) requiring combined treatment, serious laboratory examination abnormalities, and family or social factors, which will affect the safety of the subjects, or the collection of test data and samples; 16\) Patients who participated in other clinical trials of anti-tumor drugs within 28 days before enrollment.