Photo: Global Chordoma Consensus Group convenes in Milan
For people diagnosed with chordoma, the first course of treatment can shape the rest of their lives, and provides the best opportunity for long-term disease control or, possibly, a cure.
Yet because of the rarity of the disease, few doctors have significant experience treating it and care can vary widely across hospitals and regions. This can lead to suboptimal treatment, confusing or conflicting treatment recommendations, and patients and caregivers feeling that they must draw their own roadmap for high-stakes medical decisions.
To help address these challenges, a decade ago we convened a global, multidisciplinary group of leading clinicians—the Global Chordoma Consensus Group—to develop and publish the first chordoma treatment guidelines. Since their publication, these guidelines have become an invaluable reference for clinicians and patients around the world.
In that time, however, the field has continued making meaningful advances in pathology, imaging, surgery, radiation, and supportive care and generated new data about approaches that can further improve patient outcomes.
Enabling more patients to benefit from these advances is a major priority for the Chordoma Foundation. Thus, we’re excited to share that the Global Chordoma Consensus Group has just published updated consensus guidelines for evidence-based treatment of primary localized chordoma (i.e., chordoma at the time of first diagnosis that has not spread to other parts of the body).
These guidelines are intended to:
- Provide an up-to-date reference for healthcare providers who treat chordoma patients.
- Encourage providers with limited experience treating chordoma to refer patients to high volume centers capable of providing the complex, multidisciplinary care needed to effectively manage this disease.
- Provide the justification patients sometimes need to secure health-system and insurance approvals for appropriate care.
- Inform our educational resources and Patient Navigation Service, helping us provide patients with guidance that reflects the latest evidence and expert consensus.
Most importantly, they will help more patients benefit from state of the art care and have a chance to achieve the best possible outcomes.
What’s new in the guidelines
The updated guidelines reinforce one of the most important principles in chordoma care: patients should be evaluated and treated by an experienced, multidisciplinary team.
They also provide more detailed guidance than the 2015 recommendations in several key ways, including:
- Stratification by tumor location (skull base, mobile spine, and sacrum) with distinct surgical and radiotherapy guidance for each.
- Incorporation of markers like brachyury and SMARCB1/INI1 to define molecular subtypes, and centralized pathology review for diagnoses made outside an experienced center.
- More thorough imaging and pre-treatment staging recommendations.
- A role for chemotherapy in treating the more aggressive poorly differentiated and dedifferentiated subtypes.
- Follow-up surveillance tailored by subtype and age.
- Structured recommendations for palliative care and rehabilitation, addressing pain, neurological deficits, and long-term challenges.
Chordoma patients and caregivers can access the full updated guidelines within our online community, Chordoma Connections. Our free, confidential Patient Navigation Service can also help in understanding treatment recommendations, finding experienced doctors, preparing questions for appointments, and overcoming barriers to care. Our Patient Navigators are skilled at supporting patients at all stages from new diagnosis through long-term survivorship.
How this effort came together
To create these updated guidelines, we partnered with the Istituto Nazionale dei Tumori in Milan to re-convene the Global Chordoma Consensus Group, this time including over 150 leading experts (more than three times as many as were involved a decade ago!) spanning pathology, radiology, surgery, radiation oncology, medical oncology, rehabilitation medicine, and palliative care. After conducting a comprehensive review of more than 300 published studies from the past decade, the group gathered in Milan in June 2025 for a working meeting to define and discuss new recommendations. Every recommendation was assigned a level of evidence and grade of recommendation so clinicians can weigh the strength behind each one.
The consensus reached at that meeting has now been published in JAMA Oncology, making this guidance available in a high profile journal to clinicians around the world.
We appreciate our donors, who funded this effort, and the entire Global Chordoma Consensus Group for the tremendous collaboration and time commitment needed to make these guidelines possible. We’re particularly grateful to Drs. Alessandro Gronchi, Silvia Stacchiotti, Anna Maria Frezza, and Stefano Radaelli of Istituto Nazionale dei Tumori, and Dr. Giacomo Baldi of the Hospital of Prato for their leadership of the group, and deep care in synthesizing the group's recommendations.
What comes next
Importantly, the process of developing these guidelines also highlighted areas where there wasn't enough evidence to support consensus and where additional data is needed, including how to further personalize care, preserve function, improve radiation approaches, and use biomarkers to guide treatment. Answering those questions warrants continued research and focused investment.
Additionally, over the next several years we plan to facilitate efforts to develop updated guidance for additional disease settings including recurrent and advanced chordoma, and for pediatric chordoma and other unique subgroups. Eventually, we also plan to create the first chordoma-specific survivorship care recommendations.
Learn more in Boston
Advances in treatment and care will be a key topic at our upcoming International Chordoma Community Conference (for patients and family members) and Research Workshop (for researchers and clinicians), both coming to Boston this September. We hope to see you there!