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61-year-old woman with chronic back pain. Neuroimaging shows pathological fracture of the L4 vertebral body.
Chordomas are malignant bone tumours of adults found almost exclusively in the midline, most commonly in the sacrum and skull base. They are thought to arise from embryonic remnants of the notochord. Microscopically, conventional chordomas show a lobular architecture with epithelioid cells growing in nests and cords in a basophilic myxoid stroma. Cytoplasmic vacuoles impart a bubbly, “physaliferous” appearance to the tumour cells. Chondroid and dedifferentiated variants also exist. Chordomas express cytokeratins, S100, and brachyury (see Related Content). Prognosis is affected by tumour location, size, and resectability; sacral tumours generally have the longest overall survival.