Adrenocortical carcinoma (ACC) is a rare aggressive malignant tumor. According to the literature, the 5-year survival rate of ACC is 12%-47%. For patients with advanced ACC, mitotane alone or combined with traditional chemotherapy was the first-line standard treatment, but its progression-free survival was only about 1 year. However, for patients who fail the first-line treatment, there is a lack of effective treatment. For ACC patients who had failed first-line chemotherapy, a phase II clinical trial found that the objective response rate and the disease control rate of PD-1 inhibitor Keytruda were 14% and 64% respectively, and no grade 3 or 4 adverse events were observed. Anti-tumor angiogenic drugs combined with PD-1 inhibitors have shown impressive clinical data in many solid tumors. This study is aimed to evaluate the efficacy and safety of PD-1 inhibitor camrelizumab combined with apatinib in patients with recurrent or metastatic ACC after standard treatment failure, and to seek new treatment for this population.

Inclusion Criteria:

1. Histologically confirmed diagnosis of adrenocortical carcinoma;
2. Patients with metastatic or inoperable adrenocortical carcinoma that has progressed, metastasized, or recurred after first-line standard treatment (mitotane monotherapy, chemotherapy alone, mitotane combined chemotherapy);
3. Aged >=18 years;
4. Eastern Cooperative Oncology Group (ECOG) performance status 0-1;
5. At least one measurable lesion, according to RECIST 1.1;
6. Major organ functions within 28 days prior to treatment meet the following criteria(14 days without transfusion): HB≥80g/L, ANC≥1.5x10^9/L, PLT ≥80x10^9/L; TBIL≤1.5 ULN, ALT and AST ≤2.5 ULN, if there exists hepatic metastases, ALT and AST ≤5 ULN, Cr ≤1.5 ULN or CCr ≥60ml/min; INR or PT ≤1.5 ULN, APTT ≤1.5 ULN (if the patient is receiving anticoagulant therapy, PT and APTT should be within the expected treatment range); Cardiac Markers and BNP≤ULN；TSH≤ULN (If TSH is abnormal, T3 and T4 should be normal)
7. Appropriate contraception should be used from the start of treatment to 120 days after the end of treatment;
8. Have signed consent form.

Exclusion Criteria:

1. Patients with another primary malignancy within 5 years prior to starting the study drug, except for cured in situ cervical carcinoma and cured non-melanoma skin cancer;
2. Have central nervous system metastasis with symptoms and need hormonal intervention;
3. Had received strong CYP3A4 inhibitors within one week prior to enrollment or received strong CYP3A4 inducers within two weeks prior to enrollment;
4. Poor control of high blood pressure (SBP>140mmHg or DBP>90mmHg);
5. Congestive heart failure of New York Heart Association (NYHA) Class III or IV;
6. Thromboembolic events occurred within 1 year prior to enrollment;
7. ECG QT interval >500ms;
8. Previous systemic immunosuppressive therapy;
9. Previous anti-PD-1, anti-PD-L1 antibody or anti- CTLA-4 antibody treatment;
10. Received TKI treatment within 2 weeks prior to starting the study drug;
11. Participate in clinical trials of other interventional drugs within 4 weeks prior to starting the study drug;
12. Received systemic therapy with corticosteroids or other immunosuppressants within 2 weeks prior to starting the study drug;
13. An anti-tumor vaccine or a live vaccine was given within 4 weeks prior to starting the study drug;
14. Major surgery or severe trauma within 4 weeks prior to starting the study drug;
15. Severe infections occurred within 4 weeks prior to starting the study drug;
16. Have an active autoimmune disease or a history of autoimmune diseases;
17. Have a history of immunodeficiency;
18. Have an active tuberculosis infection;
19. Have active hepatitis;
20. Patients with symptoms of gastrointestinal bleeding or risk of bleeding;
21. Active infection, or patients are pregnant or breast-feeding.