Chordoma Foundation

First clinical guidelines for management of recurrent chordoma published in the Annals of Oncology

In February of 2015, patients, doctors, and caregivers gained a valuable new resource in the fight against chordoma when the first-ever evidence-based guidelines for chordoma diagnosis and treatment were published in The Lancet Oncology. Developed by an international group of more 40 chordoma experts convened by the Chordoma Foundation and the European Society of Medical Oncology (ESMO), these recommendations have quickly become the gold standard in chordoma treatment worldwide.

Now, we’re excited to share that a second set of evidence-based guidelines has been published in the Annals of Oncology detailing clinical best practices for the management of recurrent and advanced chordoma. Filling a critical gap in the literature about what to do in the case of recurrence or progression after primary treatment, these recommendations were developed by a group of more than 60 chordoma experts from the US, Europe, and Japan with support from CF and ESMO.

The driving forces behind these guidelines are Drs. Silvia Stacchiotti, Alessandro Gronchi, and Paolo Casali of the Fondazione IRCCS Istituto Nazionale dei Tumori (INT) in Milan, Italy (the National Cancer Institute of Italy). Recognizing the historical inconsistency with which chordoma patients had been treated and the need for evidence-based practices to drive better, more uniform care, they have spent the past five years working with the Chordoma Foundation to drive global consensus around treatment for this disease. Their efforts were aided by the Foundation’s late Board member and European Liaison, Hans Keulen, who helped to orchestrate two consensus-building meetings before his untimely death in 2015 due to complications from his tumor.

“It was Hans’ determination to improve care for chordoma patients that sparked this effort,” said CF Executive Director and co-author of the paper Josh Sommer. “Thanks to the leadership of our colleagues at INT and the contributions of all the physicians who lent their expertise, we have now achieved something that will have a lasting impact on the care of chordoma patients across the world: the codification of the most effective, evidence-based treatments for both newly-diagnosed and recurrent chordomas.”

Together, these two publications set the standard for optimal care of chordoma patients, and provide reliable references upon which to base educational initiatives for professionals and patients.

Treating local-regional recurrence

Local-regional recurrence – defined as “tumor relapse or progression after surgery, return of the primary tumor at the same site, or contiguous spreading of a tumor from the primary site to adjacent areas” – is a common event following initial treatment of chordoma patients, and represents a major clinical challenge.

This paper seeks to address the challenge of treating local-regional recurrences through a detailed set of recommendations about:

  • What to do if a local-regional recurrence is suspected
  • How to treat a confirmed recurrence
  • When to treat recurrences with intent to cure vs with palliative intent
  • When and how to follow up after treatment

Importantly, the paper highlights the value of a strong, multidisciplinary care team within reference centers for treatment of chordoma, and the importance of discussing the quality of life implications of various treatment options with patients.

Bringing this content to life throughout the paper are a series of flowcharts, like the one below, which walks physicians through the suggested tests, treatment strategies, and follow-up steps recommended for different recurrence scenarios.

Though the content of the paper is intended for a physician audience, we believe that all patients and family members ought to be able to access the valuable information and recommendations that it includes. To that end, the Foundation sponsored the publication of this paper in an open access format, meaning that anyone can access it for free.

Coming soon: NEW Expert Recommendations Booklet

While open access to the original paper is important, having a resource designed for patients and caregivers is even more important. That’s why the Foundation is currently in the process of translating the technical information contained in the paper into educational content designed for a lay reader.

Like the Expert Recommendations for the Diagnosis and Treatment of Chordoma booklet, this resource will be designed to empower individuals affected by chordoma to make the best care decisions possible for themselves and their loved ones.

Anticipated for release later this year, it will feature several flow chart illustrations designed to aid in decision-making about these and other questions:

  1. How can I confirm I have a recurrence?
  2. What tests should I get before treatment?
  3. What is the best treatment strategy for me based on my tumor location and treatment history?

ACCESS THE FULL ANNALS OF ONCOLOGY PAPER »

If you have any questions about the consensus paper and/or its applicability to your care journey, please contact a Chordoma Foundation Patient Navigator.