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 EasternCooperative 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:

8. hemoglobin ≥ 9g%, neutrophils> 1500 / mm3, platelets> 100 thousand / mm3

9. bilirubin ≤ 2 x upper limit of normal (UNL), Alat, Aspat ≤ 3 x UNL (iflivermetastases are present ≤ 5 x UNL)

10. creatinine clearance > 40 ml / min

11. coagulation parameters: INR, PT, APTT <1.5 x UNL (exception: patientsundergoing anticoagulation therapy, where INR, PT, APTT remain within thetherapeutic range recommended for the patient)

12. For women of reproductive age : confirmed negative pregnancy test result, and therequirement of dual barrier contraception

13. 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 aftersurgical 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 thepermitted 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 ofvitiligo, psoriasis not requiring systemic treatment, autoimmune disease of thethyroid 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: patientswith asymptomatic CNS metastases with prior surgery or radiotherapy and no historyof intracranial bleeding)

14. Circulatory failure NYHA ≥3

15. Corrected QT interval> 500 ms

16. Significant coexisting disease, including neoplastic, except for basal cellcarcinoma of the skin, carcinoma in situ: prostate, cervix, breast

17. Other significant comorbid disease that, in the investigator's opinion, would poserisks 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