The Global Chordoma Consensus Group is calling for shared decision-making, more use of molecular biomarkers for staging, and refined protocols for site-specific surgery in the care of patients with primary, localized chordoma.

Their new recommendations were written by a panel of 150 experts from a variety of specialties, including pathology, radiology, and surgery, who reviewed 305 articles from 2010 to 2025. The updated guidance replaces a 2015 consensus statement in which 40 chordoma experts participated.

The new guidance, which was published in JAMA Oncology, is more detailed than the 2015 statement, Joseph Schwab, MD, a member of the consensus group, told Medscape Medical News.

The 2015 statement “was really a review of general principles,” he said. “Whereas the most recent updated consensus statement reflects a more prescriptive, site-specific treatment algorithm.”

Chordomas, which are rare sarcomas of notochordal origin, account for about 3% of all primary bone tumors — with an incidence of less than 1 in 1 million — and for about 20% of all soft tissue and bone sarcomas, according to a 2021 consensus paper from the Connective Tissue Oncology Society. These rare malignant bone tumors have a high rate of local recurrence.

Why Update the Guidance Now?

The latest update reflects the most recent evidence surrounding the molecular nature of chordoma and the differences between conventional chordoma and poorly differentiated and dedifferentiated chordoma, Schwab said.

“So many things have changed in cancer care over the last decade that for any type of cancer you need to really rethink how you’re going to approach treating patients,” said Ziya Gokaslan, MD, chair in the Department of Neurosurgery at Brown University in Providence, Rhode Island, and a member of the consensus group.

He noted a key difference between the two statements is that the newer focuses on primary chordoma, whereas the previous document addressed both primary and metastatic chordoma.

What Are the Most Significant Changes?

The recommendations for diagnosis and staging are more detailed. While the 2015 statement recommended standard imaging and pathologic diagnosis, the latest call for more specific imaging, such as MRI of the primary tumor, whole-spine MRI and whole-body CT, and centralized pathologic review.

Gokaslan noted the 2026 consensus recommends the use of molecular markers, specifically the brachyury and SMARCB1 mutations, for characterizing disease subtypes.

“Now it’s universally accepted that you have to” confirm pathology with brachyury transcription factor, he said.

For treatment, the most significant difference in the new statement is the call to incorporate definitive radiation therapy, said Schwab, who is also director of spine oncology at Cedars-Sinai Spine Center in Los Angeles. “This is a reflection of the advances in the use of targeted radiation therapy, including stereotactic radiation, carbon ion and proton ion radiation, which have been shown to be noninferior to surgery in some cases.”

The new consensus statement also emphasized the use of high-dose radiation therapy.

“Previous experience with radiation therapy was poor, largely because the amount of radiation delivered was suboptimal,” Schwab said.

The earlier statement recommended doses below 60 Gy in most cases, whereas the latest consensus statement calls for doses of 70 Gy or greater. For example, for skull base chordoma specifically, the statement advised precise radiotherapy (RT) target volume and a dose greater than 74 Gy.

The new statement also adds clarity on when to use systemic therapy. Where the 2015 guidance stated systemic therapies had a limited role and did not recommend them for adjuvant settings, the new recommendation calls for a subtype-specific strategy.

It advises against using systemic therapy as neoadjuvant or adjuvant therapy for patients with localized disease who would benefit from surgery or RT, or both. The consensus statement also noted that systemic therapy could be considered in localized, poorly differentiated, and dedifferentiated chordoma for patients who may still have other options for treatment, Gokaslan said.

The new statement also advises following a surveillance strategy specific to subtype and age group, and provides recommendations for palliative care and rehabilitation, specifically addressing pain, neurologic deficits, and site-specific disability. The 2015 statement only offered general guidelines for follow-up and supportive care.

“Previously there was very little focus on long-term outcomes and some of the consequences, so rehab elements of palliative care had not been really fully incorporated into treatment guidelines where this particular paper more clearly articulates those elements,” Gokaslan explained.

The new statement also includes algorithms for the management of skull base, mobile spine, and sacral chordoma.

“The role of a more complete surgery with negative margins, particularly for mobile chordoma and sacral chordoma, has become better established since then, and we have had more papers coming out confirming that those patients tend to do better with morbidity,” he said. “Sometimes you’re limited and you can’t really offer that surgical procedure, then you’re relying on other treatment, particularly radiation, more. Those recommendations are incorporated into the algorithms.”

How Will Changes Affect Clinical Practice?

The consensus statement calls for a multidisciplinary approach to patient care, Gokaslan and Schwab explained.

“It increases the complexity of preoperative surgical discussion” between the patient with their surgeon, radiation oncologist, and medical oncologist, Schwab said.

For mobile spine and sacral chordoma surgery, the consensus statement requires margin documentation, said Gokaslan. “However, this is generally not feasible for skull base chordomas, for which gross total resection remains the surgical goal.”

When margin-negative resection cannot be safely achieved, Gokaslan said, the preferred strategy shifts toward planned high-dose radiation therapy, with proton or carbon ion therapy the preferred RT modalities for chordomas where available.

The updated guidance on biomarkers and treatment options provides a foundation for future direction in chordoma management, he continued.

“My hope…is that we’re going to be able to target brachyury to silence it in patients with chordomas. I think you’re going to be able to stop their disease and potentially cure them,” he said.

Radiation “is becoming more and more refined,” and with clearer guidance on the use of immunotherapy, along with genome sequencing of patients with chordoma and the identification of more molecular targets, “I think in the very near future we’ll be able to have meaningful treatment options for patients with chordoma,” Gokaslan added.

Schwab and Gokaslan report no relevant disclosures.

Richard Mark Kirkner is a medical journalist based in the Philadelphia area.