Clinical Outcomes With Notch Inhibitors in Notch-Activated Recurrent/Metastatic Adenoid Cystic Carcinoma.

ABSTRACT:

Background

Adenoid cystic carcinoma (ACC) with NOTCH-activating mutations presents a clinical challenge due to its poor prognosis. NOTCH inhibitors have emerged as a potential therapy for ACC patients with NOTCH activation. This study aimed to evaluate the efficacy of NOTCH inhibitors in this patient population.

Methods

A retrospective analysis was conducted on patients with metastatic ACC harboring NOTCH pathway activation, who received NOTCH inhibitors at MD Anderson Cancer Center. NOTCH inhibitors included AL101, a gamma-secretase inhibitor, and brontictuzumab, an antibody targeting NOTCH1. NOTCH pathway activation was assessed through genomic analysis for NOTCH-activating mutations or immunohistochemistry for NOTCH1 intracellular domain (NICD1). Efficacy endpoints includ

SUBMITTER: Hoff CO 

PROVIDER: S-EPMC11872804 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature