Study of Relacorilant in Combination With Pembrolizumab for Patients With Adrenocortical Carcinoma Which Produces Too Much Stress Hormone (Cortisol)

Inclusion Criteria:

• Histologically or cytologically confirmed ACC (advanced unresectable and/ormetastatic)
• Measurable disease based upon RECIST v1.1 as determined by the Investigator.
• Documented GC excess (too much cortisol).
• For patients who have received mitotane within 3 months prior to screening, mitotanelevels must be <4 mg/L at screening.
• Eastern Cooperative Oncology Group (ECOG) performance status ≤2.
• Adequate organ and bone marrow function (determined through blood and urine tests)
• Negative pregnancy test for patients of childbearing potential at the Screening andevery 6 weeks (+ or - 7 days) in female patients of childbearing potential.

Exclusion Criteria:

• Major surgery within 4 weeks prior to enrollment. If the participant underwent majorsurgery, they must have recovered adequately prior to starting study treatment.
• Have received and responded (complete response [CR] or partial response [PR]) to priortreatment with any prior checkpoint inhibitor or any other agents targeting T-cellstimulation pathways
• Taking a concomitant medication that is a strong Cytochrome P450 3A (CYP3A) inducer,or that is a substrate of CYP3A with a narrow therapeutic index
• Known untreated parenchymal brain metastasis or have uncontrolled central nervoussystem (CNS) metastases. Patients must not require steroids and must be neurologicallystable without corticosteroids for a minimum of 3 weeks prior to the commencement ofthe study. Patients with neurologic symptoms must undergo a CT/MRI to rule out occultCNS metastases.
• Requirement for chronic systemic GC treatment, such as active autoimmune diseaserequiring systemic treatment (corticosteroids or other immunosuppressive medication)
• Patients requiring inhaled glucocorticoids but have no other alternative treatmentoption if their condition deteriorates during the study.
• Clinically relevant toxicity from prior systemic cytotoxic therapies or radiotherapythat in the opinion of the Investigator has not resolved to NCI-CTCAE v5.0 Grade 1 orless prior to the first dose of relacorilant.
• Treated with the following prior to the first dose of relacorilant:

1. Any investigational product, systemic anticancer therapy, or radiation therapywithin 21 days
2. Antibodies or anticancer vaccines within 60 days
3. Mifepristone or other GR antagonists within 5 half-lives of these medications
4. Adrenostatic medications within 5 half-lives of these medications

• History of severe hypersensitivity to another monoclonal antibody
• Other concurrent cancer or a history of another invasive malignancy within the last 3years that has a likelihood of recurrence of >30% within the next 5 years. Adequatelytreated basal and squamous skin cancers, ductal carcinoma in situ, cervical cancer,prostate cancer, non-muscle invasive urothelial cancer or other tumors curativelytreated with no evidence of disease are permissible.
• Human immunodeficiency virus (HIV) or current chronic/active infection with hepatitisC virus or hepatitis B virus including: Chronic or active hepatitis B as diagnosed byserologic tests. In equivocal cases, hepatitis B or C polymerase chain reaction may beperformed and must be negative for enrollment.
• Clinically significant uncontrolled condition(s) or a condition which, in the opinionof the Investigator, may confound the results of the trial or interfere with thepatient's participation, including but not limited to:

1. Unstable angina pectoris, angioplasty, cardiac stenting, or myocardial infarction 3 months before study entry.
2. Active infection that requires parenteral antibiotics.
3. Has known psychiatric or substance abuse disorders that would interfere withcooperation with the requirements of the trial.