PEMBR-01: Pembrolizumab in the Treatment of Advanced, Progressive Adrenocortical Carcinoma.

Inclusion Criteria:

1. Signing the informed consent form to participate in the study
2. Age over 18 years of age
3. Histopathologically confirmed adrenocortical carcinoma
4. The general condition of the patient was assessed according to the Eastern Cooperative Oncology Group (ECOG) scale \<2
5. Measurable disease according to RECIST 1.1
6. Confirmed progression according to RECIST 1.1 within the last 6 months in patients, who received at least one line chemotherapy according to the EDP or EDP-M
7. Adequate function of the marrow and internal organs:

   1. hemoglobin ≥ 9g%, neutrophils\> 1500 / mm3, platelets\> 100 thousand / mm3
   2. bilirubin ≤ 2 x upper limit of normal (UNL), Alat, Aspat ≤ 3 x UNL (if livermetastases are present ≤ 5 x UNL)
   3. creatinine clearance \> 40 ml / min
   4. coagulation parameters: INR, PT, APTT \<1.5 x UNL (exception: patients undergoing anticoagulation therapy, where INR, PT, APTT remain within the therapeutic range recommended for the patient)
8. For women of reproductive age : confirmed negative pregnancy test result, and the requirement of dual barrier contraception
9. For men of reproductive age: the requirement of dual barrier contraception

Exclusion Criteria:

1. Pre-treatment with an immune checkpoint inhibitor
2. Any cancer therapy within the last 7 days (including mitotane)
3. Persistent side effects of previous anti-cancer therapy in the\> G1 stage or after surgical treatment (exception: alopecia)
4. Immunosuppressive therapy present or conducted within the last 4 weeks
5. Glucocorticoid therapy in a dose higher than the replacement dose (subject to the permitted use: inhaled or topical steroids, single administration of a steroid, e.g. in case of an allergic reaction to contrast, use of mineralocorticosteroids, steroids in the course of asthma or COPD)
6. Previous allograft marrow or organ transplant
7. Current or diagnosed in the last 2 years autoimmune disease with the exception of vitiligo, psoriasis not requiring systemic treatment, autoimmune disease of the thyroid gland
8. Active or previously documented inflammatory disease of the large intestine
9. Previous non-infectious pneumonia requiring steroid therapy
10. Hepatitis B or C
11. Active tuberculosis
12. Current active infection requiring systemic treatment
13. Symptomatic, untreated central nervous system (CNS) metastases (exception: patients with asymptomatic CNS metastases with prior surgery or radiotherapy and no history of intracranial bleeding)
14. Circulatory failure NYHA ≥3
15. Corrected QT interval\> 500 ms
16. Significant coexisting disease, including neoplastic, except for basal cell carcinoma of the skin, carcinoma in situ: prostate, cervix, breast
17. Other significant comorbid disease that, in the investigator's opinion, would pose risks to the patient during therapy
18. Pregnancy or breastfeeding
19. Patients requiring dialysis
20. The patient's inability to meet the requirements specified in the study protocol
21. Vaccination with live vaccine within 3 months before starting treatment