FDA Approves Ogsiveo, First-Ever Drug for Treatment of Desmoid Tumors

U.S. Food and Drug Administration

The U.S. Food and Drug Administration (FDA) recently approved Ogsiveo (nirogacestat) tablets for the systemic treatment of adult patients with progressing desmoid tumors, marking it as the first drug to be approved for this rare type of soft tissue sarcoma.

Desmoid tumors, while non-cancerous, can be locally aggressive and invade surrounding structures and organs. This invasion can result in pain, mobility issues, and a decrease in quality of life. Historically, surgical removal has been the primary treatment, but the risk of tumor recurrence or other health challenges post-surgery has led to an increased evaluation of systemic therapies.

Richard Pazdur, M.D., director of the FDA’s Oncology Center of Excellence and acting director of the Office of Oncologic Diseases in the FDA’s Center for Drug Evaluation and Research, highlighted the FDA’s commitment to addressing unmet medical needs and advancing safe and effective therapies. He stated, “Desmoid tumors are rare tumors that can lead to severe pain and disability. Today’s approval will offer the first approved treatment option for patients beyond surgery and radiation.”

Ogsiveo’s effectiveness was evaluated in an international, multicenter, randomized, double-blind, placebo-controlled trial involving 142 adult patients with progressing desmoid tumors not amenable to surgery. Patients were given either 150 milligrams (mg) of Ogsiveo or a placebo orally, twice daily, until disease progression or unacceptable toxicity. The trial showed that Ogsiveo provided a clinically meaningful and statistically significant improvement in progression-free survival compared to the placebo.

Common side effects seen in at least 15% of the patients in the trial included diarrhea, ovarian toxicity, rash, nausea, fatigue, stomatitis, headache, abdominal pain, cough, alopecia, upper respiratory tract infection, and dyspnea.

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The FDA granted the approval of Ogsiveo to SpringWorks Therapeutics Inc. under Priority Review, Fast Track and Breakthrough Therapy designations for the indication noted above, as well as Orphan-Drug designation for treatment of desmoid tumor (aggressive fibromatosis). These designations provide incentives to assist and encourage drug development for rare diseases.

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