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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 National Comprehensive Cancer Network (NCCN 2025) and the European Reference Network for Paediatric Cancer (ERN PaedCan/EURACAN/ESMO/ERN GENTURIS 2021).
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Diagnostic investigations

History and physical examination: as per NCCN 2025 guidelines, elicit history and perform physical examination in the initial evaluation of patients with chordoma.
B
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  • Imaging for staging

Diagnostic procedures

Biopsy and histopathology: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, perform biopsy in the initial work-op of patients with suspected primary bone sarcoma.
B
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Medical management

Setting of care: as per NCCN 2025 guidelines, evaluate and manage all patients with chordoma by a multidisciplinary team with expertise in the management of chordoma.
B

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Therapeutic procedures

Radiotherapy
As per NCCN 2025 guidelines:
Consider offering radiotherapy as the primary treatment in patients with unresectable conventional or chondroid chordomas.
C
Consider offering radiotherapy for positive surgical margins or for large extra compartmental tumors.
C

Surgical interventions

Surgical excision
As per NCCN 2025 guidelines:
Perform wide resection, if resectable, with or without radiotherapy as the primary treatment in patients with conventional or chondroid chordoma in the sacrococcygeal region or in the mobile spine.
B
Perform intralesional excision, if resectable, with or without radiotherapy as the primary treatment in patients with conventional or chondroid chordoma in the skull base or clival region. Obtain follow-up MRI with contrast of primary site to assess adequacy of resection. Consider performing re-resection if necessary.
B

Follow-up and surveillance

Surveillance: as per NCCN 2025 guidelines, include the following in the surveillance of patients with chordomas:
physical examination
imaging of surgical site, timing, and modality, as clinically indicated (X-ray, MRI with contrast with or without CT with contrast) for up to 10 years
chest imaging every 6 months, consider obtaining CT annually for 5 years, then annually thereafter.
B

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