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Rat sarcoma virus (RAS) proteins are a family of prototypical oncogenes frequently mutated in human cancers. Mutations in the RAS gene account for 19% of all pathogenic alterations and are the subject of extensive research in molecular and clinical oncology.1 The RAS family consists of three major isoforms, namely the Harvey rat sarcoma virus (HRAS), the neuroblastoma RAS […]

Bernd Kasper, ESMO 2022: DeFi phase 3 trial investigating nirogacestat versus placebo for progressing desmoid tumors

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Published Online: Sep 13th 2022

Desmoid tumours are rare, locally aggressive soft-tissue tumours that are challenging to manage, due to lack of approved therapies, and variable presentation. Dr Bernd Kasper (Universitätsmedizin Mannheim, Mannheim, Germany) joins touchONCOLOGY to discuss the phase 3 DeFi study, a global randomized, double-blind, placebo-controlled trial in adults with progressing desmoid tumours. The trial investigates nirogacestat, a novel oral gamma secretase inhibitor, that has shown antitumour activity in patients with desmoid tumours.

The abstract entitled ‘DeFi: A phase III, randomized controlled trial of nirogacestat versus placebo for progressing desmoid tumors‘ Abstract LBA2, was presented at European Society for Medical Oncology (ESMO) 2022.

Questions:

  1. Could you tell us a little about desmoid tumours and the unmet needs in their treatment? (0:19)
  2. What is nirogacestat and what is the rationale for its use in the treatment of desmoid tumours? (1:13)
  3. What were the aims, design and eligibility criteria of the DeFi study? (1:52)
  4. What were the efficacy and safety findings of the study? (2:40)

Disclosures: Bernd Kasper is a consultant for and receives grant/research support from Springworks; is on the advisory board for Bayer, Blueprint, Boehringer Ingelheim, Springworks, GSK, PharmaMar, and Ayala; invited Speaker for PharmaMar, Springworks, Ayala.

Support: Interview and filming supported by Touch Medical Media Ltd. Interview conducted by Katey Gabrysch.

Filmed in coverage of ESMO 2022

Access more soft tissue tumours content here

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