Global Consensus on the Management of Primary Localized Chordoma.
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Radaelli Stefano, Frezza Anna M, Fossati Piero, Baldi Giacomo G, Akiyama Toru, Asencio Jose M, Bolle Stephanie, Bovee Judith V M G, Caraceni Augusto T, Cote Gregory M, Dea Nicolas, Dei Tos Angelo P, Doglietto Francesco, Du Rebecca, Fernandez-Miranda Juan C, Ferrari Marco, Flanagan Adrienne M, Gardner Paul A, Gokaslan Ziya L, Gondi Vinai, Hall Matthew, Kelly Hillary R, Lange Nicole, Lasalvia Paolo, Lillini Roberto, Lütgendorf-Caucig Carola, MacDonald Shannon M, Martín Benlloch Juan A, Mazzatenta Diego, McKean Erin L, Mehta Minesh P, Messiou Christina, Meyer Bernhard, Morosi Carlo, Polster Sean, Redmond Kristin J, Reynolds Jeremy, Ricchini Francesca, Rosenberg Andrew, Ruppert Lisa M, Sahgal Arjun, Salvatore Daniela, Schwab Joseph H, Sciubba Daniel M, Sen Rajeev D, Snuderl Matija, Sommer Josh, Sullivan Patricia, Timmermann Beate, Trama Annalisa, Vanzulli Andrea, Vellutini Eduardo, Weber Damien C, Wedekind Mary F, Wolinsky Jean-Paul, Yamada Yoshiya, Stacchiotti Silvia, Gronchi Alessandro, Global Chordoma Consensus Group
Аннотация
OBJECTIVE: To update and expand the 2015 consensus recommendations on the diagnosis, treatment, and follow-up of pediatric and adult patients with primary, localized chordoma.
EVIDENCE REVIEW: In June 2025, a meeting of the Global Chordoma Consensus Group was held in Milan, Italy, that included experts from all relevant specialties as well as patient representatives. A comprehensive literature review guided structured discussions on the management of primary localized disease. Levels of evidence and grades of recommendation were assigned.
FINDINGS: A total of 305 articles were included in the literature review. Management strategies were stratified by anatomical site (skull base, mobile spine, and sacrum). The central principal of care was treatment at experienced, multidisciplinary centers, with maximally safe surgery followed by high-dose, highly conformal radiotherapy. Guidance was provided on diagnosis, surgical approaches, and radiotherapy planning for each anatomical site. Systemic therapy options; long-term, risk-adapted follow-up; and supportive, palliative, and rehabilitative care were also addressed.
CONCLUSIONS AND RELEVANCE: This global consensus statement provided updated multidisciplinary guidance for the management of primary, localized chordoma. It aimed to harmonize clinical practice, support shared decision-making, and identify priorities for future collaborative research in this rare and challenging disease.