AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
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Background

Neoplastic disease that involves the spine with spinal cord compression is an emergency and may be devastating if it goes unrecognized.

Primary spinal cord tumors arise from the different elements of the CNS, including neurons, supporting glial cells, and meninges. Anatomically, neoplasms of the spinal cord may be classified according to the compartment of origin, either intramedullary (inside the cord) or extramedullary (outside the cord). Additionally, cancers that metastasize to the vertebrae or surrounding tissues may cause spinal cord compression.

Although metastatic lesions are not primary neoplasms arising from neural elements of the spinal cord, they are featured in this discussion because metastases cause 85% of the cases of neoplastic spinal cord compression, and the clinical presentation tends to be indistinguishable from that of primary cancers of the spinal cord.

For the emergency physician, the cell origin of the tumor is less of a concern than the consequent syndromes of spinal cord dysfunction.

Pathophysiology

Neoplastic spinal cord compression usually follows hematogenous dissemination of malignant cells to the vertebral bodies, with subsequent expansion into the epidural space. Spread into the epidural space may occur by means of tumor extension through the intervertebral foramina or hematogenous spread by way of the Batson venous plexus. Generally, metastatic seeding appears in the thoracic spine (accounting for about 70% of cases), with the lumbar spine being the next most involved site (20% of cases). The cervical spine is affected in approximately 10% of cases. Multiple spinal levels are affected in about 30% of patients.¹

Systemic cancers with a tendency for spinal cord metastasis include the following: breast, prostate, renal, or lung neoplasms; lymphoma; sarcoma; and multiple myeloma. Gastrointestinal and pelvic malignancies tend to affect the lumbosacral spine; lung and breast cancers are more likely to affect the thoracic spine.

Most primary spinal cord tumors are astrocytomas or ependymomas. Primary tumors that affect the paravertebral area may spread and compress the cord through expansion, particularly in an intervertebral foramen. Additionally, an enlarging cancerous lymph node may compress the cord.

Metastases to the substance of the cord (intramedullary) are relatively rare. Signs and symptoms tend to simulate those of epidural compression; however, the associated motor weakness is more likely to be unilateral. Principles of treating intramedullary cancer are similar to those for epidural spinal cord compression.

Leptomeningeal metastases spread by means of diffuse or multifocal seeding of the meninges from systemic cancer (eg, lung or breast cancer, melanoma, lymphoma). Consequent signs and symptoms may be referable to the spine or spinal cord. Evidence of spinal compromise may include lower extremity weakness, paresthesias, reflex asymmetry, and spinal pain.

Hemangiomas (benign tumors of the blood vessels) are usually discovered incidentally and usually do not produce symptoms. However, symptoms emerge if pathologic vertebral fractures or epidural extension occurs.

Nerve tracts most vulnerable to mechanical pressure include the corticospinal and spinocerebellar tracts and the posterior spinal columns. Additionally, neoplasms may compromise the vascular supply, causing edema or ischemia. Less frequently, tumors may induce cyst formation or cavitation.

Frequency

United States

Metastatic lesions that involve the spinal cord affect about 5-10% of patients with cancer.² Approximately 15% of all primary CNS lesions arise from the spinal cord, with an estimated incidence rate of 0.5-2.5 cases per 100,000 population.

International

The international incidence rate parallels that of the United States.

Mortality/Morbidity

Most primary spinal cord cancers do not disseminate widely through the CNS or body. Consequent disability relates to the degree of cord impairment and anatomic level of cord injury.

• Metastatic tumors that cause epidural cord compression and dysfunction are the most common causes of oncologic CNS injury. Mortality correlates with the prognosis of the primary cancer.
• The severity of spinal cord compromise secondary to a tumor spans a wide range. Initially, symptoms may be limited to pain or minor sensory or motor disturbance. As the compression progresses, neurologic abnormalities become more pronounced, advancing to disability. Partial cord compression, such as Brown-Séquard syndrome, may evolve. In the advanced stage of compression, complete transverse sensory and motor paralysis with bowel and bladder incontinence occurs.
• The disability of the patient at the initiation of therapy serves as the best predictor of ultimate disability in patients with epidural cord compression. Therefore, early detection of cord compression and early intervention is the goal.

Sex

• A slight male predominance exists for primary spinal cord tumors.
• Symptomatic hemangiomas occur most frequently in the thoracic region of teenaged girls.

Age

People older than 50 years are more likely to experience back pain secondary to a metastatic tumor. The incidence of primary spinal cord tumors peaks in people aged 30-50 years.

• Certain CNS tumors, such as neuroblastoma, occur almost solely in pediatric patients.
• Clinical syndromes produced by intramedullary tumors vary depending on the age of the individual. In children, gait disturbances prevail, with pain reported infrequently. Spinal cord neoplasms may manifest as scoliosis or torticollis in younger patients.
• Pain is the most common early complaint of adult patients with spinal cord neoplasms, followed by the insidious progression of spinal cord dysfunction.

CLINICAL

Section 3 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

• Early symptoms are often nonspecific and include local pain or stiffness.
• Gradually worsening back pain is the classic initial feature of spinal cord neoplastic disease in about 90% of adult patients who are affected. Pain often precedes other symptoms associated with spinal cord compression by 2-4 months.
• Discomfort may be radicular, localized to the back, or both. Radicular pain suggests nerve root impingement and may be exacerbated with movement or straining.
• Pain from vertebral metastasis may worsen with a recumbent position in contrast to back pain from degenerative joint disease, which may improve with a recumbent position.
• Back discomfort that is unrelieved with rest or recumbency may also be a symptom of destructive lesions.
• A history of cancer may provide a pivotal clue in establishing the diagnosis.
• Sensory or motor symptoms that may be referred to the cord include limb paresthesias and weakness.
• Emergence of leg weakness, paresthesias in the lower extremities, and/or bowel or bladder dysfunction in patients with a history of cancer should evoke immediate concern for cord compression.
• The onset of the pain symptoms is often insidious and follows a gradually progressive course (days, weeks, months). However, once symptoms other than pain appear, symptom progression may be rapid.
• Paraplegia and bowel or bladder disturbances (eg, constipation, urinary hesitancy, retention, incontinence) are usually late findings except in conus medullaris syndrome, in which sphincter dysfunction and saddle anesthesia may emerge early in the course.

Physical

Findings on physical examination correspond to the location of the tumor, degree of cord impingement, and duration.

• Spasticity; hyperreflexia; and loss of pinprick, temperature, position, and vibratory sensation may occur early.
• Percussion tenderness over the affected spinal region may be present.
• Deep-tendon reflexes may be initially hypoactive or absent. The Babinski sign (upward movement of the toe in response to plantar stimulation) may be absent early in the course of compression.
• Pain that progresses down the asymptomatic (or less symptomatic) limb caused by straight-leg raising may suggest cord compression.
• Valsalva maneuvers, such as coughing, sneezing, or straining, may exacerbate radicular back pain from cord compression.
• Late signs include demonstrable weakness, clear sensory loss, bilateral Babinski signs, and decreased anal sphincter tone and bulbocavernosus reflex. As spinal cord compromise advances, hyperreflexia and Babinski reflexes are typically present. Lax rectal sphincter tone is a late sign of spinal cord dysfunction.
• Almost one half of patients with a tumor and subsequent spinal cord compression have some paresis, with as many as 15% of patients being paraplegic at the time of diagnosis.
• Coexisting emergence of lower extremity weakness and sensory loss may cause ataxia or a gait disturbance.
• The Lhermitte sign (ie, sudden, electric-shock–like pain with neck flexion) indicates meningeal irritation.
• Nuchal rigidity occurs in about 10% of patients with leptomeningeal metastasis.
• Partial cord disorders, such as Brown-Séquard syndrome (contralateral motor and sensory deficits), arise from lateral spinal cord compression.
• Lesions of the cauda equina and the termination of the spinal cord may cause a combination of upper motor neuron and lower motor neuron signs.
• Tumors in the region of the foramen magnum may produce quadriparesis and simulate other causes of diffuse weakness.
• If a cervical intramedullary tumor or syrinx (cavity) is present, the unusual clinical picture of isolated sensory loss may be present in the upper extremities.
• Primary tumor sites (eg, breast, lymph nodes, prostate) and/or blood in the stool may be discovered on examination.

DIFFERENTIALS

Section 4 of 11  

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• Clinical
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• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Abscess
Hematoma
Syrinx
Radiation myelopathy
Chemotherapy myelopathy
Paraneoplastic myelopathy
Neoplastic meningitis
Spinal arachnoiditis
Transverse myelitis
Central disk herniation
Spondylitic myelopathy
Disk-space infections
Diskitis

WORKUP

Section 5 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Laboratory studies are not generally helpful in establishing the diagnosis.
• Marked elevation of the erythrocyte sedimentation rate suggests infection or inflammation.
• Patients with a suspected spinal cord neoplasm are at risk for additional oncologic insults. Routine blood workup typically includes analysis of the following: CBC; prothrombin time; activated partial thromboplastin time; and metabolic profile, including calcium level and liver function.

Imaging Studies

• MRI
• • MRI of the affected area provides the best definition of spinal lesions and is the procedure of choice.
  • With MRI, the entire spine may be visualized rapidly (sagittal images), and images may be obtained in multiple planes for best definition of the lesion, vertebrae, epidural space, and spinal cord. Roughly one third of people with spinal epidural metastases have multiple spinal metastases.
  • MRI can usually be used to differentiate a collapsed vertebra secondary to osteoporosis or trauma from malignant disease.
  • The intervertebral space is usually not involved in tumors of the spine. When the disk space is obliterated, infection is more likely.
  • If MRI cannot be performed, consult a qualified radiologist or oncologist about other imaging options (eg, intrathecal contrast-enhanced myelography, CT scan, nuclear medicine bone scanning).
• Plain radiography
• • Plain radiographs may reveal bony destruction (osteolytic or osteoblastic lesions), vertebral collapse or subluxation, or calcification (associated with a meningioma). Roughly 50% of the bone must be destroyed to be visible on plain films.
  • Conventional radiographs do not provide information about spinal cord structure or compression.
  • Changes are demonstrated on plain films in about 80% of patients with spinal cord tumors. Conversely, findings on plain films are falsely negative in about 20% of cases.
• Nuclear medicine: Most tumors (excluding myeloma) exhibit increased activity on nuclear medicine scans.

Procedures

• Lumbar puncture
• • Be cautious when considering a lumbar puncture; the presence of a spinal cord tumor is a relative contraindication to the performance of a lumbar puncture.
  • Removal of cerebrospinal fluid in the presence of a tumor may worsen cord compression.
  • In leptomeningeal metastasis, examination findings of the spinal fluid are almost always abnormal and reveal elevated protein levels and positive cytologic results.
• Post-void residual urine volume: Have the patient urinate and check the urinary residual volume by catheterization when bladder impairment is a concern. Volumes greater than 200 mL may suggest a neurogenic bladder.

TREATMENT

Section 6 of 11  

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Prehospital Care

• Use of spinal immobilization precautions may be prudent when neurologic impairment is suggested.
• Support airway, breathing, and circulation during transport.

Emergency Department Care

Spinal cord compression secondary to cancer is an emergency that requires rapid diagnosis and treatment to prevent permanent complications.

• Even when a cure is not possible, timely diagnosis and treatment may improve the patient's quality of life.
• Consider administering steroids to patients who are thought to have cord compression secondary to a neoplasm.
• Chemotherapy has a limited role in treating spinal cord dysfunction and should be administered at the discretion of the consultant.
• Radiation therapy
• • Radiation treatment to areas of tumor compression should be pursued after appropriate imaging and consultation.
  • Cord compression from an epidural tumor is considered one of the few emergencies in radiation oncology.
  • Spinal cord tolerance to radiation depends on the fraction size and cumulative dose.
• Generally, patients with suspected spinal cord compression should be admitted.

Consultations

• Neurosurgeons traditionally manage spinal cord compression and dysfunction; however, local practices may vary.
• Oncology, neurology, and radiation oncology staff may be involved in some circumstances.

MEDICATION

Section 7 of 11  

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Dexamethasone administration may acutely preserve neurologic function in patients with epidural spinal cord compression. Opinions regarding dosages vary (eg, 4-100 mg q6h).

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, they modify the body's immune response to diverse stimuli. With therapy, tumor-associated edema and cord compression, particularly those caused by metastatic tumors, may diminish.

FOLLOW-UP

Section 8 of 11  

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• Follow-up
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Further Inpatient Care

• Further inpatient care may include steroid administration, chemotherapy, or surgery ordered at the discretion of attending physicians.
• Surgical decompression provides immediate relief of compression but may contribute to spinal mechanical instability. However, if instability is present from tumor destruction, surgery may be necessary for stabilization.
• Recent research suggests that a combination of surgical decompression and radiation may be more effective than radiotherapy alone. This is an area of active investigation.³
• Treatment is individualized and depends on tumor type, degree of neurologic function, and other factors.

Further Outpatient Care

• At the direction of the attending physicians, outpatient care may include ongoing chemotherapy, steroid administration, radiation therapy, or other treatments.
• Physical therapy may be necessary.
• Hospice care may be the best choice in some cases.

Transfer

• Transfer may be necessary when specialized services are not accessible at the initial site of evaluation.
• Consider administering steroids prior to transfer in cases of suspected spinal cord impairment caused by tumor.

Complications

• Paraplegia
• Quadriplegia
• Urinary tract infections
• Soft-tissue damage
• Respiratory complications

Prognosis

• The prognosis for recovery of neurologic deficits secondary to spinal cord compression is related to the duration and severity of the impairment at the start of treatment.
• Disturbances in sphincter function are associated with a poor prognosis for recovery.
• Primary CNS spinal cord neoplasms are usually not metastatic and generally have a more favorable prognosis for long-term survival than do metastases.
• Patients with leptomeningeal metastases have a poor prognosis.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Patients with spinal cord compression may have normal findings at neurologic examination, and pain or subjective sensory disturbances may be the only complaint. Pay particular attention to any past medical history of cancer, which should increase the suspicion for metastatic disease.
• Failure to perform an adequate neurologic examination is a frequent pitfall. In patients with pertinent symptoms, examination might include testing for perineal sensation, muscle stretch reflexes, and plantar reflexes. Spastic reflexes associated with higher cord involvement may be absent with lesions of the cauda equina and conus medullaris.
• Pay particular attention to symptoms involving the bladder or bowel, especially incontinence or difficulty voiding or defecating. Postvoid residual volume may be a sign of neurogenic bladder.
• Obtain an MRI when spinal cord impingement is suspected. Failure to do so on an emergent basis is a common pitfall. MRI is often ordered by specifying vertebral levels, while neurologic disability is defined by spinal cord levels. When ordering MRI, be sure to specify the region that includes the area of spinal cord dysfunction.
• Epidural metastasis may be the initial manifestation of a malignancy. Epidural metastasis may occur in the following sites (in descending order of frequency): lung, unknown primary site, prostate, renal cell, breast, and colorectal carcinoma. Ensure timely follow-up care for back pain that does not respond to conservative therapy.

Special Concerns

• Tumors or other compressive lesions near the foramen magnum may be difficult to diagnose. Symptoms that resemble those of amyotrophic lateral sclerosis with muscle wasting and hyperreflexia may also confound the diagnosis.

MULTIMEDIA

Section 10 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Patient with metastatic breast cancer; plain radiograph shows L4 vertebral collapse.
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Media type:  X-RAY

Media file 2:  MRI of plain film above showing intrusion of tumor and vertebral collapse into spinal canal.
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Media type:  MRI

Media file 3:  Patient with renal cell carcinoma; MR shows collapse of a thoracic vertebra with spinal cord impingement.
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Media type:  MRI

Media file 4:  Axial MR of patient in Media File 3 above with vertebral destruction and spinal cord impingement.
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Media type:  MRI

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

1. Spinazze S, Caraceni A, Schrijvers D. Epidural spinal cord compression. Crit Rev Oncol Hematol. Dec 2005;56(3):397-406. [Medline].
2. Prasad D, Schiff D. Malignant spinal-cord compression. Lancet Oncol. Jan 2005;6(1):15-24. [Medline].
3. Regine WF, Tibbs PA, Young A. Metastatic spinal cord compression: a randomized trial of direct decompressive surgical resection plus radiotherapy vs radiotherapy alone. Int J Radiat Oncol Biol Phys. 2003;57 (suppl 2):5125.
4. Gilbert MR, Minhas TA. Epidural spinal cord compression and neoplastic meningitis. In: Johnson RT, ed. Current Therapy in Neurologic Disease. 1997:253-9.
5. Newton HB, Shah SML. Neurological syndromes and symptoms in the cancer patient: differential diagnosis, assessment protocols, and targeted clinical interventions. Emerg Med Rep. 1997;18:149-58.
6. Patten J. The spinal cord in relation to the vertebral column. In: Neurologic Differential Diagnosis. 1996:247-81.
7. Sansur CA, Pouratian N, Dumont AS, Schiff D, Shaffrey CI, Shaffrey ME. Part II: spinal-cord neoplasms--primary tumours of the bony spine and adjacent soft tissues. Lancet Oncol. Feb 2007;8(2):137-47. [Medline].
8. Schiff D. Spinal cord compression. Neurol Clin. Feb 2003;21(1):67-86, viii. [Medline].
9. Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. Neurology. Oct 1996;47(4):906-12. [Medline].
10. Schiff D, O'Neill BP, Suman VJ. Spinal epidural metastasis as the initial manifestation of malignancy: clinical features and diagnostic approach. Neurology. Aug 1997;49(2):452-6. [Medline].
11. Schmidt RD, Markovchick V. Nontraumatic spinal cord compression. J Emerg Med. Mar-Apr 1992;10(2):189-99. [Medline].
12. Traul DE, Shaffrey ME, Schiff D. Part I: spinal-cord neoplasms-intradural neoplasms. Lancet Oncol. Jan 2007;8(1):35-45. [Medline].

Article Last Updated: Sep 17, 2007