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Chordoma — SCE Medical Oncology MCQ

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HardCNS TumoursChordomaSCE Medical Oncology

A 45-year-old woman presents with a slowly growing skull base mass causing cranial nerve palsies. Biopsy confirms a chordoma (positive for brachyury). What is the key treatment challenge and approach?

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Correct answer: D — Chordomas are resistant to conventional-dose radiotherapy; surgery (en-bloc resection) combined with high-dose proton beam therapy achieves the best local control

Chordomas are rare notochordal tumours arising from the skull base or sacrum. They are characterised by brachyury expression, slow growth and high local recurrence. They are resistant to conventional radiotherapy but respond to high-dose proton beam therapy (≥70 Gy RBE) due to the sharp Bragg peak allowing dose escalation while sparing adjacent critical structures (brainstem, optic chiasm). Surgery (maximal safe resection) followed by proton therapy is the standard approach. The UK proton beam therapy centres (Christie/UCLH) treat chordomas.

Reference: NICE; ESMO 2024 Rare Tumour Guidelines; NHS England Proton Beam Therapy Programme