- Department of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
- Department of Orthopaedics, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
Ekkehard M. Kasper
Department of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
DOI:10.4103/2152-7806.139673Copyright: © 2014 Lam FC. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Lam FC, Arle JE, Glazer PA, Kasper EM. Primary Extradural Tumors of the Spine – Case Review with Evidence-guided Management. Surg Neurol Int 28-Aug-2014;5:
How to cite this URL: Lam FC, Arle JE, Glazer PA, Kasper EM. Primary Extradural Tumors of the Spine – Case Review with Evidence-guided Management. Surg Neurol Int 28-Aug-2014;5:. Available from: http://sni.wpengine.com/surgicalint_articles/primary-extradural-tumors-of-the-spine-case-review-with-evidence-guided-management/
Background:Primary extradural tumors of the spine comprise only a small percentage of all spinal tumors. However, given their relative radioresistance and their typical malignant, invasive nature, surgery may be associated with fairly high morbidity and mortality rates. Furthermore, it may be especially difficult to achieve gross total resections with tumor-free margins.
Case Descriptions:We present two cases and review the literature regarding the presentation, diagnosis, and evidence-based guidance/treatment of primary extradural spinal tumors. The first patient with an L1 chordoma presented with cauda equina syndrome. Following surgery, the second patient, with a giant cell tumor of the cervicothoracic junction, responded well to the novel drug denosumab, a monoclonal antibody to the RANK ligand.
Conclusion:Primary extradural spine tumors pose significant challenges to surgeons as the aim is to achieve satisfactory surgical outcomes with clean tumor margins (e.g, thus avoid recurrence) while minimizing morbidity. Improvements in radiotherapy, chemotherapy, and novel molecular drugs may increase survival rates and improve overall outcomes.
Keywords: Chemotherapy, extradural, malignant, molecular drug treatments, primary, radiotherapy, spine, tumors
Primary extradural tumors of the spine are rare and constitute approximately 4% of all spine tumors.[
Case 1 - L1 Chordoma
A 53-year-old male who presented with acute urinary incontinence and right leg numbness, exhibited saddle anesthesia, decreased rectal tone, and numbness in the right L2 distribution on physical examination. Sagittal T2-weighted magnetic resonance imaging (T2W MRI) of the lumbar spine showed a hypointense, nonenhancing intraosseous lesion at L1 [
a) 53-year-old male presenting with an L1 chordoma. b) Sagittal T2WI shows a hyperintense lesion of L1 with a large exophytic component compressing the conus on axial T1WI. c) Postoperative sagittal X-ray showing L1 vertebrectomy with T11 to L3 pedicle screw and rod fixiation. d) Axial CT through L1 shows insertion of a carbon fiber cage packed with autologous bone graft
Case 2: Giant cell tumor of the cervicothoracic junction
A 38-year-old female presented with several months of neck pain. On physical examination, she only demonstrated midline cervicothoracic tenderness; otherwise she was neurologically intact. Sagittal [
38-year-old female with a T1 giant cell tumor. a) Sagittal and b) axial T2WI of the cervical spine shows a hyperintense lesion at T1 eroding through the posterior cortex causing spinal cord compression. c) Postoperative sagittal CT scan of the spine showing T1 interbody fusion and plating with posterior C5 to T3 instrumentation and fusion. d) axial T2WI through T1 showing an anterior strut graft with a generous decompression of the spinal canal
Chordomas are slow growing, locally aggressive lesions that can occur anywhere along the axial skeleton, but are most commonly found at the craniocervical junction and the sacrum.[
Conventional radiation therapy has little effect on chordomas, but may still be offered as palliation for subjective pain control and to delay the time to recurrence.[
Giant cell tumors
GCTs comprise 5% of all adult primary bone tumors and occur most frequently at the epiphyseal–metaphyseal junction of long bones. They most commonly involve the sacrum, followed by the cervical and thoracic regions. Although they are aggressive benign tumors, their size and vascularity make resection difficult; therefore, patients may require preoperative embolization to decrease intraoperative blood loss.
Surgical management of spine GCTs involving the thoracic and lumbar spine should include en bloc resection to decrease the rate of local recurrence. Intralesional resection can provide adequate control for Enneking stage II tumors. Cryotherapy, phenolization, and bone cement may be used as adjuvant treatments to wall off the resection cavity. Patients should be monitored for at least 5 years for disease recurrence.[
There is currently very low quality evidence supporting the role of radiation for treatment of recurrent GCTs.[
A recent clinical trial using denosumab, a monoclonal antibody to the RANK ligand to modulate osteoclast activation, showed a response rate of 86% and functional improvement with decreased pain in 84% of patients.[
Primary extradural spine tumors are rare, and there is little high quality evidence outlining the optimal treatment for these lesions. Trends in the literature support en bloc spondylectomy for low grade malignant tumors, but this aggressive approach proves technically challenging and may be associated with relatively high morbidity and mortality rates. Improvements in radiotherapy technology, advances in chemotherapy, novel molecular drug targets, and other multimodality protocols may increase survival rates.
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