TY - JOUR
T1 - Global Consensus on the Management of Primary Localized Chordoma
AU - Global Chordoma Consensus Group
AU - Radaelli, Stefano
AU - Frezza, Anna M
AU - Fossati, Piero
AU - Baldi, Giacomo G
AU - Akiyama, Toru
AU - Asencio, Jose M
AU - Bolle, Stephanie
AU - Bovee, Judith V M G
AU - Caraceni, Augusto T
AU - Cote, Gregory M
AU - Dea, Nicolas
AU - Dei Tos, Angelo P
AU - Doglietto, Francesco
AU - Du, Rebecca
AU - Fernandez-Miranda, Juan C
AU - Ferrari, Marco
AU - Flanagan, Adrienne M
AU - Gardner, Paul A
AU - Gokaslan, Ziya L
AU - Gondi, Vinai
AU - Hall, Matthew
AU - Kelly, Hillary R
AU - Lange, Nicole
AU - Lasalvia, Paolo
AU - Lillini, Roberto
AU - Lütgendorf-Caucig, Carola
AU - MacDonald, Shannon M
AU - Martín Benlloch, Juan A
AU - Mazzatenta, Diego
AU - McKean, Erin L
AU - Mehta, Minesh P
AU - Messiou, Christina
AU - Meyer, Bernhard
AU - Morosi, Carlo
AU - Polster, Sean
AU - Redmond, Kristin J
AU - Reynolds, Jeremy
AU - Ricchini, Francesca
AU - Rosenberg, Andrew
AU - Ruppert, Lisa M
AU - Sahgal, Arjun
AU - Salvatore, Daniela
AU - Schwab, Joseph H
AU - Sciubba, Daniel M
AU - Sen, Rajeev D
AU - Snuderl, Matija
AU - Sommer, Josh
AU - Sullivan, Patricia
AU - Timmermann, Beate
AU - Trama, Annalisa
PY - 2026/7/9
Y1 - 2026/7/9
N2 - IMPORTANCE: Chordoma is a rare malignant bone tumor with high local recurrence, metastatic spread in 40% to 60% of patients over the disease course, and significant morbidity. Because of its rarity, anatomical complexity, and prolonged natural history, high-quality evidence to guide management is limited. International consensus guidelines for localized chordoma were first published in 2015; however, advances in pathology, imaging, surgery, radiotherapy, and supportive care since then necessitate updated multidisciplinary recommendations.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.
AB - IMPORTANCE: Chordoma is a rare malignant bone tumor with high local recurrence, metastatic spread in 40% to 60% of patients over the disease course, and significant morbidity. Because of its rarity, anatomical complexity, and prolonged natural history, high-quality evidence to guide management is limited. International consensus guidelines for localized chordoma were first published in 2015; however, advances in pathology, imaging, surgery, radiotherapy, and supportive care since then necessitate updated multidisciplinary recommendations.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.
U2 - 10.1001/jamaoncol.2026.2054
DO - 10.1001/jamaoncol.2026.2054
M3 - Article
C2 - 42424068
SN - 2374-2437
JO - JAMA Oncology
JF - JAMA Oncology
ER -