A rare case of extradural thoracic spinal chordoma with medullary compression and literature review

Abstract

Background. Chordomas are low-grade, slow-growing, but locally invasive and aggressive malignant tumors, arising from the remnants of the embryonic notochord. Given its origin, the distribution of chordomas is along the axial skeleton: 50% of cases in the sacral region, 35% skull base and 15% occur in the vertebral bodies of the spine, which most commonly involve cervical (particularly C2), followed by lumbar, and then the thoracic spine. Without a prompt and aggressive treatment the chances of metastasis are low, but the local recurrence rate is high (about 50%) with a 40% chance of 10-year survival. Objective. Our purpose is to present a rare case of an extradural thoracic chordoma, the importance of differential diagnosis and the management of this case for a better outcome. Case presentation. A 61-year-old man presented with a 1-month history of thoracic back pain, followed by rapidly progressive paraparesis and paresthesia in both lower limbs, and 24 hour urinary retention. MRI of the thoracic spine revealed an contrast-enhancing lesion arising from T5 to T6 vertebral level, with pre and paravertebral extension and medullary compression. The diagnosis of chordoma was made by tissue biopsy and histopathological study. There are few reports of chordomas with this localization in English literature. After surgery, the patient showed slightly good clinical outcome in terms of improvement of muscle strength in lower limbs and back pain decrease in intensity of back pain. Conclusions. Primary chordoma in the thoracic spine is extremely rare (less than 15% of the cases). This case highlights the importance of emergency surgery in the treatment of spinal tumors and the need to consider chordoma as a differential diagnosis for extradural spinal tumors. On the other hand the treatment is challenging because the current literature is lacking in evidence based guidelines due to low incidence.

Keywords

• chordoma
• extradural spinal tumors
• intramedullary compression
• rapidly progressive paraparesis