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Brachyury Chordoma — SCE Medical Oncology MCQ

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HardSarcomaBrachyury ChordomaSCE Medical Oncology

A 55-year-old man with dedifferentiated chordoma of the sacrum has exhausted conventional treatment options (surgery + proton RT). An emerging targeted approach targets a protein highly expressed in chordomas. What is this target?

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Correct answer: E — Brachyury (TBXT) — a transcription factor pathognomonic for chordoma; brachyury-targeting therapeutic vaccines and T-cell-based therapies are in clinical trials

Brachyury (encoded by TBXT gene) is a T-box transcription factor virtually pathognomonic for chordoma. Brachyury drives chordoma biology and is an attractive therapeutic target. Approaches: brachyury-targeting vaccines (e.g. BN-brachyury), brachyury-directed CAR-T cells, and CDK4/6 inhibitors (CDK4 amplification common in chordoma). No approved targeted therapy yet; clinical trial enrolment encouraged.

Reference: ESMO Rare Tumour; Brachyury; Chordoma Foundation