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Nirogacestat

Gamma secretase inhibitor

Table of contents

What is nirogacestat used for?

Nirogacestat blocks the enzyme gamma secretase.


It treats desmoid tumours by slowing down or stopping growth of the tumours.

What is the mechanism of action of nirogacestat?

Tumour growth signals that lead to desmoid tumours come from, when a body process known as the notch pathway is overactive.

The notch pathway requires the enzyme gamma-secretase to work properly.

By blocking gamma-secretase, this stops the tumour growth signals.
This can slow tumour growth, reduce pain, and improve quality of life.

What are the contraindications of nirogacestat?

At the time of writing, there are no known conditions that prevent taking nirogacestat according to its full prescribing documentation.

Important nirogacestat prescribing safety information.

The action of nirogacestat  on the notch pathway affects intestines, phosphate levels, and ovarian function.

As a result nirogacestat may cause diarrhoea, low phosphate levels, and ovarian issues in women.

So regular monitoring through blood tests and hormone checks are advised as they can help manage side effects early. 

Nirogacestat patient counselling advice.

Patients should be advised to avoid grapefruit and Seville oranges as they can interfere with how nirogacestat breaks down in the body.

Advise patients to seek medical attention if they experience severe diarrhoea, signs of dehydration, or any concerning changes in their reproductive function.

Recommend maintaining a healthy lifestyle that includes staying well-hydrated, a balanced diet and adequate sleep.

References

1. Gounder, M.M., Ravin Ratan, Alcindor, T., Schöffski, P., Ta, Wilky, B.A., Riedel, R.F., Lim, A., L Mary Smith, Moody, S., Attia, S., Chawla, S.P., D’Amato, G.Z., Federman, N., Merriam, P., Van, B.A., Vincenzi, B., Benson, C., Bui, N.Q. and Chugh, R. (2023). Nirogacestat, a γ-Secretase Inhibitor for Desmoid Tumors. The New England Journal of Medicine, 388(10), pp.898–912. doi:https://doi.org/10.1056/nejmoa2210140. Link


2. Loggers, E.T., Chugh, R., Federman, N., Hartner, L., Riedel, R.F., Cho, S., Hyslop, D., Lim, A., Oton, A.B. and Oktay, K.H. (2024). Onset and resolution of ovarian toxicity with nirogacestat treatment in females with desmoid tumors: Updated safety analyses from the DeFi phase 3 study. Cancer. doi:https://doi.org/10.1002/cncr.35324. Link


3. Messersmith, W.A., Shapiro, G.I., Cleary, J.M., Jimeno, A., Dasari, A., Huang, B., Shaik, N., Cesari, R., Zheng, X., Reynolds, J.L., English, P.A., McLachlan, K., Kern, K.A. and LoRusso, P. (2015). A Phase I, Dose-Finding Study in Patients with Advanced Solid Malignancies of the Oral γ-Secretase Inhibitor PF-03084014. 21(1), pp.60–67. doi:https://doi.org/10.1158/1078-0432.ccr-14-0607. Link

 

4. OGSIVEO® (nirogacestat) -. (2023). Desmoid Tumor Information, Resources, and Support - OGSIVEO® (nirogacestat). [online] Available at: https://www.ogsiveo.com [Accessed 29 Aug. 2024]. Link