In a recent paper in the Journal of Pathology, Researchers at the University College London report that approximately 60% of chordomas have amplification (extra copies) of an important cancer gene that codes for the Epidermal Growth Factor Receptor (EGFR). EGFR is a signaling protein that drives cell growth and which has been implicated in the development of a many types of cancer. Several drugs (Erbitux, Iressa and Tarceva) that inhibit the activity of the EGFR protein are approved for treating lung cancer, colon cancer and others.

In addition to being amplified in the majority of Chordomas, the EGFR protein was found to be activated in all of the chordoma tumors studied, as well as the U-CH1 chordoma cell line. Furthermore, when researchers treated the U-CH1 cell line with an EGFR inhibitor they found that it shut down the activated EGFR, and caused the cells to stop growing.

Taken together, these findings provide strong evidence for the use of EGFR inhibitors to treat chordoma patients with amplified or activated EGFR. It may also explain several case reports in the literature of chordoma patients responding to treatment with EGFR inhibitors (see summary of published treatment results). Molecular analysis of EGFR is routinely performed in many hospitals, and in light of these recent findings might be warranted to help select systemic therapy for chordoma patients with progressive disease.

This study was carried out in the lab of Dr. Adrienne Flanagan, one of the world’s premier bone pathologists and a member of the Chordoma Foundation Scientific Advisory Board. The Chordoma Foundation provided the U-CH1 cell line used for this research to Dr. Flanagan’s lab.