Giant cell tumour of bone involving C2 with pathological hangman\'s fracture

Clinical Cases 11.10.2014
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 53 years, female
Authors: Ramírez-Fuentes C, Elía I, Navarro V, Atarés M
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Clinical History

A 53-year-old woman presented to the emergency department with a two-week history of cervical pain, left upper extremity paresthesias and dysphagia. On physical examination, cervical flexion and soft tissue swelling was noted. The patient was afebrile and no trauma was referred. Routine laboratory tests were normal.

Imaging Findings

Lateral cervical spine radiography showed C2-C3 anterolisthesis with a lytic lesion in the body and odontoid process of the axis.

CT confirmed the presence of a lytic lesion of the axis with absence of mineralization and aggressive features, such as cortical destruction, expansive remodelling and an associated soft-tissue mass. The tumour was located in the vertebral body and the odontoid process with involvement of both pedicles and left lamina. There was a displaced fracture through the pars interarticularis of C2 with significant angulation and anterior displacement at C2-C3 (pathological hangman’s fracture). CT angiography showed stretching of the left vertebral artery between fracture fragments.

On MR images, the tumour had low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2 and STIR. There was an associated paraspinal haematoma.

Thoracic and abdominopelvic CT was normal. A bone biopsy was performed and GCT could be confirmed.

Discussion

Giant cell tumours (GCT) of bone are relatively common bone tumours, usually benign and typically found in the metaepiphysis of long bones. GCT tumours involving the spine are rare and cervical localization is even more uncommon, representing less than 1% of GCT tumours [1].

They are typically seen in early adulthood, with 80% of cases occurring between 20 and 50 years of age. Only 13% of cases occur in patients over the age of 50 years [2]. The clinical GCT spine symptoms are pain, weakness, and sensory deficits. Associated vertebral fracture is often apparent.

Pathologically, giant cell tumour consists of abundant osteoclastic giant cells intermixed throughout the spindle cell stroma.

On radiography and CT, spinal GCT has been reported to be an expansile lytic lesion without a sclerotic rim. Generally the tumour has a tendency to have low signal intensity on both T1 and T2 weighted MR images, because of the haemosiderin deposition and dense collagen matrix within the tumour [3].

The tumour usually originates in the vertebral body and extends to the posterior elements in advanced lesions. While the epiphyseal-metaphyseal location of GCT in the long bone is a clue to the radiographic diagnosis, these landmarks are not available in the spine, so it is difficult to ensure the correct definitive diagnosis [3].

In this case, the presence of a single bone lesion in a patient over 50 years raises the differential diagnosis between metastasis, solitary plasmacytoma and primary bone tumour. Some atypical features made the definitive diagnosis of GCT difficult, such as the rare location of the tumour, the age of presentation and the high-signal intensity on T2-weighted images on MR due to pathological fracture and haemorrhage. The biopsy of the lesion remained mandatory for the final diagnosis.

This patient was treated conservatively with cervical orthesis and denosumab, because surgical resection was likely to result in severe morbidity.

Classically, GCT of bone has been treated surgically with curettage and placement of cement or bone graft, with recurrence rates relatively high, ranging from 15% to 25% [4]. In recent years, the new chemotherapeutic denosumab has been used to treat advanced or unresectable GCT of bone. The drug is a monoclonal antibody that targets the RANKL (receptor activator of nuclear factor κB ligand), stopping the osteoclastic activity of cells in GCT and reducing bony destruction [5]. In the near future, denosumab may offer a treatment option as neoadyuvant to facilitate the surgery or even to replace it.

Differential Diagnosis List

Giant cell tumour of bone involving C2 with pathological fracture.
Metastasis
Myeloma
Chordoma

Final Diagnosis

Giant cell tumour of bone involving C2 with pathological fracture.

Liscense

Figures

Lateral cervical spine radiography

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Lateral cervical spine radiography

Sagittal CT reformat

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Sagittal CT reformat

Coronal CT reformat

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Coronal CT reformat

Axial CT images

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Axial CT images

Axial CT angiography

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Axial CT angiography

Percutaneous CT-guided bone biopsy

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Percutaneous CT-guided bone biopsy

Radiological follow-up

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Radiological follow-up

MR imaging

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MR imaging