Progressing desmoid tumors

Active Ingredient: Nirogacestat

Indication for Nirogacestat

Population group: only adults (18 years old or older)
Therapeutic intent: Curative procedure

Nirogacestat as monotherapy is indicated for the treatment of adult patients with progressing desmoid tumours who require systemic treatment.

For this indication, competent medicine agencies globally authorize below treatments:

150 mg twice daily until disease progression or unacceptable toxicity

For:

Dosage regimens

Oral, 150 milligrams nirogacestat, 2 times daily.

Detailed description

The recommended dose is 150 mg nirogacestat twice daily, one dose in the morning and one dose in the evening. This dose should not be exceeded.

Duration of treatment

Nirogacestat should be continued until disease progression or unacceptable toxicity.

Missed dose

If a dose of nirogacestat is missed, patients should not take an additional dose. Patients should take the next prescribed dose.

Dose adjustments for adverse reactions

For severe adverse reactions, or in the event of life‑threatening adverse reactions, nirogacestat should be withheld until the reaction is resolved to Grade ≤ 1 or baseline. Nirogacestat should only be restarted at a dose of 100 mg twice daily and only after carefully considering the potential benefit and likelihood of recurrence of the adverse reaction. Nirogacestat should be permanently discontinued for recurrence of severe or life-threatening adverse reaction upon rechallenge at the reduced dose.

Elderly population

No dose adjustment is recommended for patients who are aged 65 years or over. Clinical data in patients aged 65 years or over is limited.

Dosage considerations

Patients should avoid consuming grapefruit and grapefruit juice while taking nirogacestat.

Active ingredient

Nirogacestat

Nirogacestat is a reversible and non‑competitive inhibitor of gamma secretase that blocks proteolytic activation of the Notch receptor.

Read more about Nirogacestat

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