Chordoma Foundation

First clinical guidelines for chordoma treatment published in The Lancet Oncology

This month, chordoma patients, caregivers, and physicians got a powerful new resource to aid in the fight against chordoma with the publication of the first-ever set of detailed, multidisciplinary expert recommendations for the appropriate treatment of chordoma.

The guidelines, published in the leading clinical oncology research journal The Lancet Oncology, are the outcome of a year-long global effort spearheaded by Drs. Silvia Stacchiotti and Paolo Casali of the Fondazione IRCCS Istituto Nazionale dei Tumori (INT) in Milan, Italy and Chordoma Foundation Board member, Hans Keulen. INT is Italy’s main referral center for the treatment and study of cancer.

In December 2013, at the invitation of Drs. Casali and Stacchiotti, 40 chordoma experts representing multiple disciplines from both sides of the Atlantic convened in Milan during a European Society of Medical Oncology conference to launch this ambitious effort to codify the most effective, evidence-based treatments for chordoma. The Chordoma Foundation provided funding for this meeting.

“Chordoma is a very rare tumor, but in the last 10 years much progress has been made in the understanding of the biology of this disease, and, as a result, new therapeutic opportunities have been introduced,” Stacchiotti said. “However, it remains a challenging disease to manage and requires a multidisciplinary approach with coordinated involvement of several specialists. We see a great deal of inconsistency in how chordoma patients are treated, resulting in suboptimal outcomes for many patients. Therefore, it was evident that global consensus around the management of this tumor was needed.”

Meeting participants agreed on the need to take a multidisciplinary approach to chordoma and to build an international community of experts and patients to share their diverse perspectives. Throughout 2014, the group drafted and edited the treatment guidelines and prepared them for publication in one of the world’s foremost peer-reviewed medical journals. “Building a global consensus approach to chordoma: a position paper from the medical and patient community,” appeared in the February 2015 edition of The Lancet Oncology.

The clinical guidelines offer a level of detail unprecedented for chordoma, addressing factors such as:

  • Location of the tumor (skull base, spine, or sacrum)
  • Point in time (newly diagnosed, recurrent, etc.)
  • Modes of treatment (surgery, radiation and systemic therapy)

“Until now, there had never been a consensus laid out by medical experts on how chordoma should be treated,” said Chordoma Foundation Executive Director Josh Sommer, who provided a patient perspective and co-authored the paper. “This is a significant achievement because it will empower patients and families around the world to make wise treatment decisions, and will provide physicians with a road map to most effectively care for their chordoma patients.”

The publication of these treatment guidelines marks a major step forward in the Chordoma Foundation’s efforts to improve the lives of patients by working with the medical community to improve the diagnosis and treatment of this rare cancer. This paper is just the start, Stacchiotti noted. “More still needs to be done to address open questions, especially for recurrent disease, and to improve the level of evidence supporting each recommendation. We hope that in the near future we can meet again to gain consensus on these additional topics.”

To access the consensus paper that appeared in The Lancet Oncology, click here or email us at support@chordoma.org. Because this paper has been written for a physician audience, the Foundation plans to translate the report into educational content more appropriate for a lay audience.