Teaching Case

A 56-year-old female patient presented with low-back and hip pain radiating down to her left leg for 12 months. She did not respond to initial management with conservative therapy of non-steroidal anti-inflammatory medications.

MRI (magnetic resonance imaging) of the lumbar spine (3T scanner) was performed using T2-weighted, T1-weighted, SPAIR, and fat-saturated contrast-enhanced T1-weighted sequences.
Figure 1. T2-weighted sagittal MRI of lumbar spine showing bone-based malignancy from L3 to L5 causing severe canal stenosis and infiltrating anteriorly into the retroperitoneal space.
Figure 2. T2-weighted axial magnetic resonance image show very high signal intensity of the tumour, which infiltrating posteriorly into the paraspinal musculature and expanding into the abdomen and psoas muscles bilaterally. The tumour causes severe spinal stenosis with compression of the nerves.
Figure 3. Sagittal T1-weighted image shows a low-intensity lesion penetrating from vertebral bodies to the retroperitoneum.
Figure 4. Sagittal T2-SPAIR image shows a well-circumscribed high-intensity lesion.
Figure 5. T1-weighted image with gadolinium contrast demonstrates heterogenous contrast-enhancement of the tumour.
MRI exam presented the mass exhibited homogeneous low-intensity on T1-weighted images; hyperintensity in the intraosseous and extraosseous component on T2-weighted images, compared with the normal vertebral body. The mass extended into the spinal canal posteriorly and perivertebral space anterolaterally infiltrating the psoas muscles and penetrating to the retroperitoneal space with surrounding the abdominal aorta for less than half the circumference. After intravenous administration of contrast material, the heterogeneous enhancement was observed.

Background: Chordoma is a rare tumour that arises from notochord remnants in the vertebral column and base of the skull, and presented as slow-growing, locally-invasive malignant tumours. It most commonly occurs in the base of the cranium or the sacrococcygeal region but around 15-20% affect the vertebral body. Chordomas typically affect those in the 40-60-year-old age group but have been reported in children and the very elderly. [1,2,3]
Clinical perspective: It is a slow-growing tumour, which makes it difficult to diagnose and follow up after treatment. Affected patients usually have a poor prognosis because of the extensive nature of the disease at the time of diagnosis, which may make it difficult to perform a radical resection. [1,3,4]
Imaging perspective: On CT, chordoma appears as a well-circumscribed lytic lesion with, sometimes with marginal sclerosis and central calcifications. It may exhibit moderate to marked enhancement following contrast administration. Currently, MRI is the preferred imaging modality for pre-operative diagnosis and assessment. Chordomas have lower signal intensity on T1-weighted imaging and may show foci of hyperintensity which represents intratumoural haemorrhage. T1-weighted imaging with gadolinium contrast demonstrates heterogenous contrast-enhancement of the tumour with a honeycomb appearance. On T2-weighed imaging chordomas tend to be hyperintense. Gradient-echo MRI/SWI imaging can confirm intratumoural haemorrhage. [2,3,4,5]
Outcome: The treatment which provides the longest survival is complete en bloc resection of the tumour with clean margins. In the majority of cases, margin-free excision is difficult due to surrounding neurovascular structures and extensive tumour size. Most physicians advocate for radiation therapy after any type of chordoma resection and recommend lifelong surveillance with magnetic resonance imaging due to the high local recurrence rate. Chordomas are relatively radioresistant necessitating high-dose radiation therapy. [1,3,4,5,6]
Teaching points: Most of the chordomas exhibit very high signal on T2-weighted imaging.

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