Table of contents
Chordoma
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of chordoma are prepared by our editorial team based on guidelines from the European Reference Network for Paediatric Cancer (ERN PaedCan/EURACAN/ESMO/ERN GENTURIS 2021) and the National Comprehensive Cancer Network (NCCN 2021).
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Diagnostic investigations
History and physical examination: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, elicit medical history and perform physical examination in the initial work-up of patients with suspected primary bone sarcoma.
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Imaging for staging
Diagnostic procedures
Medical management
Setting of care: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, manage patients with bone sarcomas at reference centers and/or within reference networks able to provide access to the full spectrum of care and age-specific expertise.
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Chemotherapy
Management of metastatic disease
Therapeutic procedures
Radiotherapy: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, offer definitive radiotherapy (without debulking) in the following cases:
inoperable patients
R0 or R1 resection cannot be achieved according to an expert center
neurological impairment not accepted by the patient.
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Surgical interventions
Follow-up and surveillance
Surveillance: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, consider obtaining follow-up approximately every 3 months for the first 2 years, every 6 months for years 3-5, every 6-12 months for years 5-10, and every 0.5-1-2 years thereafter.
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Management of recurrent disease