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Chordoma

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 on Genetic Tumour Risk Syndromes (GENTURIS/ERN PaedCan/EURACAN/ESMO 2021) and the National Comprehensive Cancer Network (NCCN 2021).
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Guidelines

1.Diagnostic investigations

History and physical examination: as per ESMO 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

2.Diagnostic procedures

Biopsy and histopathology: perform biopsy in the initial work-op of patients with suspected primary bone sarcoma.
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3.Medical management

Setting of care: as per ESMO 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

4.Therapeutic procedures

Radiotherapy: as per ESMO 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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5.Surgical interventions

Surgical excision: as per ESMO 2021 guidelines, consider performing surgery in patients with a chordoma arising from S4 and below or in the context of other alternatives for tumors originating above S3.
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6.Follow-up and surveillance

Surveillance: as per ESMO 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