A PHASE 2 TRIAL OF NIROGACESTAT IN PATIENTS WITH RECURRENT OVARIAN GRANULOSA CELL TUMORS

This phase 2 clinical trial will study the effectiveness of nirogacestat in ovarian granulosa
cell tumors (OvGCTs). Nirogacestat is a gamma secretase inhibitor (GSI) which is hypothesized
to decrease the growth and activity of ovarian granulosa tumors.

Key Inclusion Criteria:

- Has histologically confirmed recurrent adult-type granulosa cell tumor of the ovary
prior to first dose of study treatment

- Have documented radiological evidence of relapse after at least one systemic therapy
that is not amenable to surgery, or radiation and have measurable disease by RECIST
v1.1 criteria

- Have adequate bone marrow, renal and hepatic function as defined by screening visit
laboratory values

Key Exclusion Criteria:

- Has signs of bowel obstruction requiring parenteral nutrition, malabsorption syndrome
or preexisting gastrointestinal conditions that may impair absorption of nirogacestat

- Has had a major cardiac or thrombo-embolic event within 6 months of signing informed
consent

- Has abnormal QT interval at Screening, or has congenital or acquired long QT syndrome
or a history of additional risk factors for Torsades de Pointes

- Has current or chronic history of liver disease or known hepatic or biliary
abnormalities

- Has received bevacizumab treatment or other monoclonal antibody therapy with targeted
anti-angiogenic activity for OvGCT within 28 days (or 5 half-lives, whichever is
shorter) prior to the first dose of study treatment;

- Has received treatment for OvGCT including but not limited to the following within 28
days (or 5 half-lives, whichever is longer) prior to the first dose of study
treatment: hormonal therapy, chemotherapy, immunotherapy, targeted therapy or any
investigational treatment