The NCCN has updated their guidelines for soft tissue sarcoma to include the γ-secretase inhibitor nirogacestat as a systemic therapy option for patients with desmoid tumors, granting the agent a preferred, category 1 recommendation.
Desmoid tumors are noncancerous growths that appear in connective tissue, most often on the arms, legs, and abdomen. Aggressive tumors must be treated with surgery, radiation, or chemotherapy to keep them from growing into nearby organs, which can make them life-threatening. They can cause pain and disfigurement, and they can reduce functioning and quality of life.
The FDA approved Ogsiveo (nirogacestat) tablets for adult patients with progressing desmoid tumors who require systemic treatment. Nirogacestat is a new molecular entity and the first drug to be approved for the treatment of patients with desmoid tumors, a subtype of soft tissue sarcomas.
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