This phase II trial studies the side effects and how well nirogacestat works in treating patients less than 18 years of age with desmoid tumors that has grown after at least one form of treatment by mouth or in the vein that cannot be removed by surgery. Nirogacestat may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.
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Progression-free survival (PFS)
Timeframe: From initiation of treatment to occurrence of disease progression or death from any cause, assessed up to 2 years
Incidence of adverse events
Timeframe: Up to 2 years
Pharmacokinetic (PK) parameter: systemic exposure
Timeframe: Up to Cycle 3 (each cycle lasts 28 days)
PK parameter: drug clearance
Timeframe: Up to Cycle 3 (each cycle lasts 28 days)
PK parameter: half-life
Timeframe: Up to Cycle 3 (each cycle lasts 28 days)