Sacral Chordoma

Clinical Cases 11.04.2005
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 71 years, female
Authors: Robinson G, Britton J
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Details
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AI Report

Clinical History

A 71-year-old female patient presented with a four-month history of increasing difficulty with defecation and micturition and a swelling around her right elbow. On examination, it was found that the swelling was fixed to deep tissue.

Imaging Findings

A 71-year-old female patient presented with a four-month history of increasing difficulty with defecation and micturition and a swelling around her right elbow. On examination, it was found that the swelling was fixed to deep tissue. Her past medical history included the diagnosis of a sacral chordoma made eleven years earlier. This had been treated with subtotal excision and radiotherapy. Nine years later, she had represented with further bladder problems, and the imaging study performed at that time had revealed the chordoma to have locally recurred (Fig. 1). A further de-bulking operation had been performed. On this presentation, an MRI and a CT scan of her sacrum and arm were performed (Figs. 2–4) . The results of these findings showed that the sacral chordoma had once again increased in size, with an evidence of more bone destruction. The swelling in the arm was shown to be a large soft-tissue mass involving the triceps muscle with multiple fluid and solid compartments. Radiologically, it had similar features to the sacral chordoma. The differential diagnosis of this mass included a soft-tissue sarcoma or a metastasis from the chordoma. The subsequent histological study confirmed the latter.

Discussion

Chordomas are rare, generally slow-growing malignant tumours, with 50% of them originating in the sacrum. Most sacral chordomas present with pain and at diagnosis are locally extensive which, given the proximity of vital structures, make the treatment options challenging and controversial. These include total excision, subtotal excision, radiotherapy and chemotherapy. Some studies advocate, where possible, radical surgery, which may increase the symptom-free interval before relapse. However, in many tumours this may not be surgically possible, and in addition it carries with it a high risk of morbidity. Therefore, a subtotal debulking procedure is often carried out. The adjuvant radiotherapy procedure remains controversial and can be used pre- or post-operatively, which may increase the remission period. The chordomas can commonly recur locally and more rarely with distant metastases. Local recurrence figures are quoted between 19% and 82% with most studies indicating figures towards the latter end of this spectrum. Distant metastases occur in 14%–30% of the cases, most commonly to the bones, the lung and the subcutaneous tissue. However, deposits in the brain, the pericardium, the ovary and the synovium have been described. Metastases to the skeletal muscle are rare and may be misdiagnosed as primary soft-tissue neoplasms. Their incidence is low in both patients with and without known malignancy, and the skeletal muscle itself is thought to be relevantly resistant to both primary and secondary cancer. In our case, the appearance of the metastatic deposit, despite being non-specific, was radiologically similar to the primary chordoma. This case highlights both that sacral chordomas can develop distant metastases, and that rare combinations of disease do exist. However, it is unlikely that the true incidence is known, as many such cases may well go unreported.

Differential Diagnosis List

Recurrent sacral chordoma, metastatic to the skeletal muscle.

Final Diagnosis

Recurrent sacral chordoma, metastatic to the skeletal muscle.

Liscense

Figures

A sagittal T2W MRI scan of the lumbar spine

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A sagittal T2W MRI scan of the lumbar spine

A sagittal T2W MRI of the lumbar spine and an axial CT scan of the sacrum (bone windows)

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A sagittal T2W MRI of the lumbar spine and an axial CT scan of the sacrum (bone windows)
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A sagittal T2W MRI of the lumbar spine and an axial CT scan of the sacrum (bone windows)

An axial T2W MRI at the level of the mid shaft of the humerus

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An axial T2W MRI at the level of the mid shaft of the humerus

A sagittal T1W post-contrast scan through the humeral mass

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A sagittal T1W post-contrast scan through the humeral mass