Malignant Transformation of Thoracic Spinal Chordoma
Keywords:
dedifferentiated chordoma, cord compression, thoracic spine, tumour recurrenceAbstract
Chordoma of the thoracic spine is an uncommon low-grade malignant tumour of bone. It can undergo transformation into a sarcoma (dedifferentiated chordoma), characterised by an aggressive, clinical course. Sarcomatous transformation is thought to stem from exposure to therapeutic radiation although recent experience suggests it can occur de-novo, or at the site of tumour recurrence. Most dedifferentiated chordomas arise from the sacrococcygeal spine and only three elderly patients were reported to suffer from this form of chordoma of the thoracic spine. This case report is of interest as the disease affected a 15-year-old-boy. He presented with progressive paraparesis. Computed tomography revealed a bone-eroding tumour at T4, characteristic of a chordoma. Despite radical excision and adjuvant radiotherapy, it was discovered he had metastasis in bone and lymph nodes five months later. The tumour had probably dedifferentiated into a sarcoma.
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