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/or
   metastatic)

   - 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, mitotane
   levels 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 and
   every 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 major
   surgery, they must have recovered adequately prior to starting study treatment.

   - Have received and responded (complete response [CR] or partial response [PR]) to prior
   treatment with any prior checkpoint inhibitor or any other agents targeting T-cell
   stimulation 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 nervous
   system (CNS) metastases. Patients must not require steroids and must be neurologically
   stable without corticosteroids for a minimum of 3 weeks prior to the commencement of
   the study. Patients with neurologic symptoms must undergo a CT/MRI to rule out occult
   CNS metastases.

   - Requirement for chronic systemic GC treatment, such as active autoimmune disease
   requiring systemic treatment (corticosteroids or other immunosuppressive medication)

   - Patients requiring inhaled glucocorticoids but have no other alternative treatment
   option if their condition deteriorates during the study.

   - Clinically relevant toxicity from prior systemic cytotoxic therapies or radiotherapy
   that in the opinion of the Investigator has not resolved to NCI-CTCAE v5.0 Grade 1 or
   less 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 therapy
      within 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 3
   years that has a likelihood of recurrence of >30% within the next 5 years. Adequately
   treated basal and squamous skin cancers, ductal carcinoma in situ, cervical cancer,
   prostate cancer, non-muscle invasive urothelial cancer or other tumors curatively
   treated with no evidence of disease are permissible.

   - Human immunodeficiency virus (HIV) or current chronic/active infection with hepatitis
   C virus or hepatitis B virus including: Chronic or active hepatitis B as diagnosed by
   serologic tests. In equivocal cases, hepatitis B or C polymerase chain reaction may be
   performed and must be negative for enrollment.

   - Clinically significant uncontrolled condition(s) or a condition which, in the opinion
   of the Investigator, may confound the results of the trial or interfere with the
   patient'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 with
      cooperation with the requirements of the trial.