- Medical Scientist Training Program, Johns Hopkins School of Medicine, Baltimore, Maryland
- Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, Maryland
- Johns Hopkins Spinal Column Biomechanics and Surgical Outcomes Laboratory, Baltimore, Maryland
DOI:10.4103/2152-7806.63911© 2010 Bydon A 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 work is properly cited.
How to cite this article: Xu R, Sciubba DM, Gokaslan ZL, Bydon A. Metastasis to the occipitocervical junction: A case report and review of the literature. Surg Neurol Int 31-May-2010;1:16
How to cite this URL: Xu R, Sciubba DM, Gokaslan ZL, Bydon A. Metastasis to the occipitocervical junction: A case report and review of the literature. Surg Neurol Int 31-May-2010;1:16. Available from: http://sni.wpengine.com/surgicalint_articles/metastasis-to-the-occipitocervical-junction-a-case-report-and-review-of-the-literature/
BackgroundThe management of metastatic spinal disease is generally considered palliative, as the progression of systemic disease is likely to hinder survival. Although the occurrence of C1-C2 instability due to metastatic disease is not uncommon and thus treatment options have been well-defined, craniocervical instability due to lesions occurring at the junction of the occiput and atlas is more rare, and treatment for metastasis to this region is less well-defined.
Case DescriptionWe present a patient with non-small-cell lung cancer metastatic to the atlanto-occipital facet joint complex. A drastic improvement in the presenting debilitating mechanical neck pain was noted following an occipitocervical fusion. A literature review of published cases of metastases to the occipitocervical junction was conducted along with treatment options.
ConclusionsThe atlanto-occipital facet joint is a rare site of metastatic disease. Destruction of this joint can lead to significant neck pain secondary to instability. Spinal fusion may afford significant and rapid resolution of these symptoms, and should be considered in the management of patients—even those with end-stage oncologic disease.
Keywords: Cervical, craniocervical, instability, metastasis, occipital
Lung cancer is classified by the World Health Organization as the leading cause of cancer-related deaths in men and the second highest in women, claiming more than 1.2 million lives worldwide per year.[
The prognosis of patients diagnosed with lung cancer is poor, due to association with systemic, incurable disease.[
NSCLC metastasizes to a variety of locations within the body, including the brain, bone, liver, and adrenal glands, in order of decreasing prevalence.[
In this report, we present a patient with known NSCLC metastasis who presented with severe axial neck pain due to tumor infiltration and destruction of the atlanto-occiptal joint. Although the etiologies and treatment strategies for managing destructive lesions of the atlanto-axial (C1-C2) joint are known, to our knowledge, this is the first published case of an NSCLC lesion that metastasized to both the occiptal condyle and the atlas (atlanto-occipital joint), causing instability and severe neck pain. We also review the literature and discuss current treatment options for bony metastasis to the occipitocervical junction.
A 66-year-old female with a two-year history of NSCLC status post lobectomy, with metastasis to the right kidney, right femur and pelvis, presented with severe occipital headaches and debilitating neck pain. She was functionally independent, with a Karnofsky score of 90. She was neurologically intact except for a left homonymous hemionopsia due to an old posterior cerebral artery distribution infarct. Brain and cervical spine computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed an old right occipital stroke, right C1 lateral mass and occipital condyle destructive lytic lesions, and a fracture of the right atlanto-occipital joint [Figure
Computed tomography (CT) and magnetic resonance imaging (MRI) of the atlanto-occipital junction. a. An axial CT image shows significant hypodense areas in the right occipitocervical junction, demonstrating extensive tumor infiltration. b. The coronal CT image again illustrates the scope of metastatic disease in both the right atlas and occipital condyle, with both being almost entirely consumed by the tumor. c. A sagittal view shows hypodense destructive lytic masses in both the occipital condyle and atlas. d. A T2-weighted MR image shows normal cerebral spinal fluid distribution with no evidence of spinal cord compression
The patient underwent an occiput to C5 instrumented stabilization and fusion using an occipital plate connected to lateral mass screws at C3, C4, and C5 [Figures
A postoperative sagittal cervical spine CT shows the placement of occiput to C5 instrumentation. (Note that the metastatic NSCLC lesion at the C0-C1 joint was not resected during surgery and that the subsequent alleviation of pain in the patient was achieved through mechanical stabilization of the occipitocervical joint alone)
Distant bony metastases of lung cancer have been associated with significant skeletal-related events (SREs), including pain (secondary to either tumor infiltration or ensuing mechanical instability), pathological fractures, hypercalcemia, and spinal cord compression.[
Metastatic involvement of the craniovertebral junction due to other primary tumors has been described in seven cases with documentation of involvement of the C0-C1 junction and ensuing instability [
Given the poor prognosis of metastatic spine disease, especially late-stage NSCLC, treatment for such lesions are palliative in nature. Well-defined NSCLC lesions may be treated with radiotherapy and/or surgery.[
Although survival of patients with metastatic spinal disease ultimately depends on the extent and control of the underlying systemic disease, quality of life can be substantially improved with surgical stabilization. In their landmark study, Patchel et al. concluded that direct surgical decompression plus radiation therapy for metastatic epidural spinal cord compression improved functional status, relative to ambulation and continence, compared to radiation alone.[
In most cases, patients with metastatic disease at the atlanto-occipital joint experience a reduction in headache or upper neck pain following occipitocervical fusion.[
This is the first reported case of NSCLC metastasizing to the atlanto-occipital joint causing severe sudden onset neck pain due to mechanical instability. Significant alleviation of pain was achieved via surgical stabilization (occipitocervical fusion). Although metastatic disease caused by NSCLC carries a poor prognosis, occipitocervical stabilization may provide excellent pain relief and improvements in quality of life in properly selected patients.
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