Clinical Features and Diagnosis of Neoplastic Epidural Spinal Cord Compression - UpToDate

2/19/2019 Clinical features and diagnosis of neoplastic epidural spinal cord compression - UpToDate
Authors: David Schiff, MD, Ilya Laufer, MD, Mark Bilsky, MD
Section Editors: Reed E Drews, MD, Patrick Y Wen, MD
Deputy Editor: April F Eichler, MD, MPH
Contributor Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jan 2019. | This topic last updated: Oct 30, 2018.
INTRODUCTION
Neoplastic epidural spinal cord compression (ESCC) is a relatively common complication of cancer
that can cause pain, mechanical instability of the spine, and potentially irreversible loss of neurologic
function. Early recognition and diagnosis of ESCC facilitates definitive and palliative therapies,
which aim to maximize function, treat pain, and prevent further neurologic complications.
The epidemiology, pathophysiology, clinical features, and diagnosis of ESCC will be reviewed here.
The treatment and prognosis of this disorder are discussed separately. (See "Treatment and
prognosis of neoplastic epidural spinal cord compression".)
TERMINOLOGY
The degree of thecal sac compression required for the designation of ESCC has been variably
defined. Throughout this topic, the term ESCC is used to mean any radiologic evidence of
indentation of the thecal sac, whether or not there are neurologic signs and symptoms associated
with compression (figure 1) [1,2].
In adults, the tip of the spinal cord usually lies at the L1 vertebral level; below this level, the
lumbosacral nerve roots form the cauda equina, which floats in cerebrospinal fluid (CSF). Since the
pathophysiology of compression of the thecal sac at the level of the cauda equina does not differ
significantly from that of more rostral compression, compression of the cauda equina is still
generally referred to by the slightly inaccurate name of ESCC.
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EPIDEMIOLOGY
ESCC is a relatively common complication of cancer. In populationbased studies, the annual
incidence of symptomatic ESCC is approximately 3 to 5 cases per 100 patients dying from cancer
[35].
Although metastatic tumors from any primary site can cause ESCC, approximately half of cases
arise from common cancers such as prostate, lung, and breast, which have a propensity to involve
and metastasize to bone. Among over 15,000 hospitalizations for ESCC in the United States from
1998 to 2006, the most prevalent underlying cancers were lung cancer (25 percent), prostate cancer
(16 percent), multiple myeloma (11 percent), and breast cancer (7 percent) [4]. Cancerspecific
annual incidence rates among patients dying from cancer were highest for multiple myeloma (15 per
100), Hodgkin and nonHodgkin lymphoma (6 to 8 per 100), prostate and kidney (5 per 100), and
thyroid (2 per 100).
ESCC is the initial manifestation of malignancy in approximately 20 percent of patients [6,7]. The
distribution of primary tumors in this setting differs from that seen in patients with ESCC and known
cancer. Synchronous presentations are more common in lung cancer, cancer of unknown primary
site, multiple myeloma, and nonHodgkin lymphoma, whereas ESCC is usually a later complication
of advanced breast and prostate cancer [6].
Only a small proportion of vertebral metastases are complicated by ESCC. At autopsy, vertebral
metastases have been described in 90 percent of patients dying with prostate cancer, 74 percent
with breast cancer, 45 percent with lung cancer, 29 percent with lymphoma or renal cell carcinoma,
and 25 percent with gastrointestinal cancers [8].
The tumors responsible for ESCC in children are different. Sarcomas (especially Ewing sarcoma)
and neuroblastomas are the most frequent causes, followed by germ cell neoplasms and Hodgkin
lymphoma [9].
PATHOPHYSIOLOGY
Spinal cord compression is a function of spinal anatomy. The spinal cord is enclosed by a protective
ring of bones comprised of the vertebral body anteriorly, the pedicles laterally, and the lamina and
spinous process posteriorly. Within this ring is the thecal sac, the outermost layer of which is
comprised of dura. Between the bone and dura lies the epidural space, which normally contains fat
and the venous plexus. (See "Anatomy and localization of spinal cord disorders".)
At each spinal level, nerve roots exit lateral to the spinal cord and posterior to the vertebral body
(figure 2). ESCC occurs when tumor invades the epidural space and compresses the thecal sac.
The degree of thecal sac compression can range from a minor asymptomatic indentation on the
normal thecal sac contour to strangulation of the spinal cord with associated paraplegia.
Approximately 85 to 90 percent of cases of ESCC are due to metastatic tumor in the vertebral
bones, but the mechanism of metastasis varies. Potential mechanisms include the following:
Arterial seeding of bone probably accounts for most cases.
For pelvic tumors, particularly prostate cancer, Batson venous plexus probably plays an
important role. When abdominal pressure is increased by the Valsalva maneuver, venous
drainage from the abdomen and pelvis is shunted to the epidural venous plexus, which
promotes vertebral metastases.
In approximately 10 percent of cases, a paraspinal mass gains access to the epidural space via
the neural foramen; this scenario is particularly common with lymphoma.
Rarely, tumor appears to emanate from the epidural space without a bony or paraspinal
component.
When bone is the source of an epidural mass, the vertebral body is involved in over 80 percent of
cases, with involvement of the posterior elements being much less common. Thus, the bulk of tumor
is anterior or anterolateral to the thecal sac in most cases of ESCC [8,10].
As tumor grows in the epidural space, it generally takes the path of least resistance and encircles
the thecal sac. As the epidural venous plexus becomes obstructed, vasogenic edema may develop
in the white matter and eventually the gray matter of the spinal cord. The beneficial actions of
glucocorticoids in ESCC may be related to resolution of vasogenic edema. If the epidural tumor is
unchecked, spinal cord infarction eventually ensues [11].
CLINICAL FEATURES
The cardinal clinical features of ESCC are symptomatic spinal cord or nerve root compression and
mechanical instability of the spinal column. Early diagnosis of spinal metastases prevents the
development of neurologic deficits and severe structural instability.
Clinical presentation — The majority of patients with ESCC have pain as the initial symptom, prior
to the onset of motor or bladder dysfunction. Delayed recognition and therapy of ESCC may result in
the development or progression of neurologic deficits.
ESCC most commonly arises in the thoracic spine. Approximately 60 to 70 percent of cases occur in
the thoracic spine, 20 to 30 percent in the lumbosacral spine, and 10 percent in the cervical spine
[12]. These percentages are in rough proportion to the combined volumes of the vertebral bodies in
each region.
In older series, most patients with newly diagnosed ESCC were not ambulatory at diagnosis [5,13
18]. Fortunately, wide availability of magnetic resonance imaging (MRI) facilitates early diagnosis of
ESCC and has led to a significantly decreased proportion of patients presenting with loss of
ambulation at the time of diagnosis [19]. (See "Treatment and prognosis of neoplastic epidural
spinal cord compression", section on 'Importance of early detection'.)
Symptoms and signs
Pain — Pain is usually the first symptom of ESCC, present in 80 to 95 percent of patients at the
time of diagnosis [5,8,20]. On average, pain precedes other neurologic symptoms of ESCC by
seven weeks.
Affected patients usually notice a severe local back pain, at the level of the lesion, which
progressively increases in intensity. However, referred pain from ESCC is common and can be
misleading in terms of localizing the lesion. One example is thoracolumbar junction (T11L1)
vertebral metastases manifesting as pain in the lower lumbar spine or in the area of the sacroiliac
joint. Another example is compression of ascending spinal cord tracts in the cervical cord
manifesting as sciatic pain; this phenomenon is sometimes called funicular pain [21].
Pain from ESCC is often worse at night, possibly related to diurnal variation in levels of endogenous
corticosteroids [22]. Local pain may be due to disruption of the periosteum or dural nerves, the
spinal cord, or paravertebral soft tissue. The frequent alleviation of pain with corticosteroids
suggests that inflammation or neural irritation plays a significant role [8].
Over time, the pain may develop a radicular quality. It may, for example, radiate into a limb with
movement of the spine or Valsalva maneuver. Radicular pain is more common in lumbosacral
lesions than in thoracic lesions [20]. Thoracic radicular pain is commonly bilateral and wraps around
anteriorly in a bandlike fashion. Abrupt worsening of pain may herald a pathologic compression
fracture.
Pain present only on movement suggests mechanical spinal instability, a finding that may demand a
surgical approach for optimal relief of pain [2325]. The movements that elicit mechanical pain vary
according to the spinal level of the tumor [26,27]:
Unstable cervical metastases cause neck and/or interscapular pain with
flexion/extension/rotation of the neck.
Unstable thoracic metastases cause pain while lying down.
Lumbar mechanical instability is generally manifested through a mechanical radiculopathy, a
severe radicular pain elicited by axial loading while ambulating or standing.
The Spine Instability Neoplastic Score (SINS) was developed in order to aid in the detection of
potential spinal instability and to facilitate decisions about surgical evaluation (table 1) [28]. (See
"Treatment and prognosis of neoplastic epidural spinal cord compression", section on 'Mechanical
(spinal stability)'.)
Motor findings — Motor findings represent advanced stages of ESCC [14,20]. The severity of
weakness tends to be greatest in patients with compressive thoracic metastases [20]. Unless there
is profound weakness or severe pain with movement, the motor examination must include standing
and walking to be complete.
When the site of compression is at or above the conus medullaris, weakness is from corticospinal
tract dysfunction and has the typical pyramidal pattern, preferentially affecting the flexors in the
lower extremities (ie, weakness of hip flexion, knee flexion, and ankle dorsiflexion with relative
preservation of hip extension, knee extension, and plantar flexion strength). If compression is above
the thoracic spine, the extensors of the upper extremities may also be affected (ie, more
pronounced weakness in the triceps and wrist extensors than in the deltoid and biceps).
Hyperreflexia below the level of the compression and extensor plantar responses may be seen.
ESCC at or above the conus medullaris generally produces fairly symmetric lower extremity
weakness. A laterally situated epidural lesion may preferentially affect a nerve root exiting the neural
foramen and produce a superimposed or isolated motor radiculopathy. Compressive lesions at the
level of the cauda equina tend to produce more asymmetrical and patchy weakness. (See 'Cauda
equina syndrome' below.)
The progression of motor findings prior to diagnosis of ESCC typically consists of increasing
weakness followed sequentially by loss of gait function and then paralysis [20]. In advanced stages
of ESCC, loss of ambulation is usually due to weakness. However, neoplastic spinal mechanical
instability may cause severe pain with movement, thereby rendering patients unable to ambulate in
the absence of neurologic deficits.
Sensory findings — Sensory findings are less common than motor findings but are still present
in a majority of patients at diagnosis [20]. Patients frequently report a pattern of ascending
numbness and paresthesias if questioned and examined carefully.
When a spinal sensory level is present, it is typically one to five levels below the actual level of cord
compression [8]. Saddle sensory loss is commonly present in cauda equina lesions, while lesions
above the cauda equina frequently result in sparing of sacral dermatomes to pinprick.
Sensory loss can occur in a radicular distribution. It has been suggested that radicular pain with
sensory complaints is particularly common with lumbar ESCC, whereas bilateral leg weakness with
back pain is typical of thoracic ESCC [20].
Lhermitte phenomenon, the experience of electricity down the spine with neck flexion, may
occasionally be reported when compression is in the cervical spine, although it is not specific for
ESCC. Lhermitte phenomenon is more commonly seen in multiple sclerosis, cervical spondylotic
myelopathy, cisplatininduced neurotoxicity, radiationinduced myelopathy, and neck trauma [29,30].
(See "Cervical spondylotic myelopathy" and "Complications of spinal cord irradiation".)
Bladder and bowel dysfunction — Bladder and bowel dysfunction due to ESCC is generally a
late finding that was present in as many as onehalf of patients in older series [20]. Urinary retention
as a manifestation of autonomic dysfunction is the most common finding and is rarely the sole
symptom of ESCC [8].
One exception is when the metastasis compresses the spinal cord at the level of the conus
medullaris, which often causes back pain and urinary/bowel symptoms with no motor or sensory
findings. Sacral metastases may also cause isolated bowel/bladder incontinence and perineal
sensory deficits due to localized compression of the sacral nerve roots.
The opiates that patients with ESCC frequently require for pain management can also contribute to
urinary retention.
Ataxia — New gait ataxia in the setting of back pain in a cancer patient should raise suspicion of
ESCC [31]. In the absence of demonstrable sensory loss, spinocerebellar tract dysfunction has
been presumed to account for the ataxia.
Cauda equina syndrome — Patients with compressive lesions in the lumbosacral spine below
the level of the conus medullaris may present with signs and symptoms of cauda equina syndrome.
Symptoms are due to compression of multiple nerve roots of the cauda equina and may include pain
radiating into one or both legs (radicular pain), paresthesias and sensory loss in the distribution of
one or more nerve roots (figure 3), and patchy and asymmetrical weakness and loss of reflexes
(figure 4). Bowel and bladder dysfunction may occur with compression of the lower sacral nerve
roots.
Weakness tends to be less severe and asymmetrical with ESCC in the lumbosacral region
compared with thoracic lesions. Cauda equina syndrome is not specific for ESCC, and
leptomeningeal metastases (carcinomatous meningitis) must also be considered, particularly in
patients with known metastatic cancer and preexisting central nervous system metastases. (See
"Clinical features and diagnosis of leptomeningeal metastases from solid tumors", section on
'Radiculopathy and cauda equina syndrome'.)
Imaging features and grading — MRI with and without contrast provides the most accurate
evaluation of the extent of disease within the thecal sac and of involvement of adjacent soft tissues
and bone (image 1). T1 without gadolinium and fatsuppressed T2 sequences provide optimal
visualization of osseous infiltration by tumor. T1 with gadolinium and T2 sequences provide optimal
visualization of epidural and paraspinal tumor extension.
Axial T2weighted images at the level of the lesion are used to assign an ESCC score, which is
widely used to describe the extent of epidural tumor [32]. The system consists of four grades (figure
1):
Grade 0 – Tumor confined to bone (not considered ESCC).
Grade 1 – Tumor with epidural extension without contact with the spinal cord or just spinal cord
abutment without displacement.
Grade 2 – Tumor that displaces or compresses the spinal cord, without circumferential tumor
extension or obliteration of the cerebrospinal fluid (CSF) space.
Grade 3 – Tumor with circumferential epidural extension and/or that causes severe spinal cord
compression with obliteration of the CSF space.
Grades 2 and 3 represent highgrade spinal cord compression, whereas grade 1 represents low
grade ESCC. (See "Treatment and prognosis of neoplastic epidural spinal cord compression",
section on 'Neurologic (high versus lowgrade ESCC)'.)
Computed tomography (CT) of the spine does not demonstrate the spinal cord or epidural space
clearly, even when intravenous contrast is used. In patients without severe osteoporosis, it can
depict metastatic disruption of the bony cortex surrounding the spinal canal, a finding that is highly
predictive of epidural tumor extension. Although CT retains some utility in conjunction with
myelography, its use has largely been replaced by MRI. (See 'Patients who cannot undergo MRI'
below.)
DIAGNOSTIC EVALUATION
The diagnosis of ESCC depends upon the demonstration of a neoplastic mass that extrinsically
compresses the thecal sac. Careful imaging of the thecal sac and epidural space is mandatory for
early diagnosis and optimal treatment planning.
MRI of the entire spine — Magnetic resonance imaging (MRI) of the entire spine is the preferred
modality for the initial evaluation of a patient with suspected ESCC. Imaging should be obtained as
soon as possible and within 24 hours in patients suspected of having ESCC [33].
Rationale and protocol — Wholespine imaging is important in patients with ESCC for two main
reasons:
Bone metastases are rarely limited to a single site, and multiple epidural tumor deposits are
present in approximately onethird of patients with ESCC [2,3436]. The presence of multiple
sites of disease significantly affects both prognosis and treatment planning [2,34].
Clinical localization is imprecise for spinal cord levels. The compressive lesion may be several
levels higher than what is suggested by a sensory level. (See 'Sensory findings' above.)
Intravenous contrast should be given for optimal evaluation of the extent of epidural and paraspinal
tumor and to evaluate for the presence of intramedullary or leptomeningeal enhancement that may
suggest an alternative or more disseminated process. (See 'Other neurologic complications of
cancer' below.)
Because complete spine imaging with axial views at every level is time consuming and
uncomfortable in patients with back pain, some institutions have developed shorter protocols for
complete spine imaging in patients with suspected ESCC that are limited to sagittal T2weighted
and T1weighted postcontrast images of the entire spine. Axial T2 and postcontrast T1 images are
obtained only at levels with abnormal findings on sagittal imaging.
In some instances when recumbent pain prevents the patient from lying still for either MRI or
myelography, a bolus of glucocorticoids may relieve pain sufficiently to allow the procedure.
The importance of imaging the entire thecal sac is illustrated by a retrospective study of 337 cases
[2]. In this report, failure to image the cervical spine in patients with symptomatic thoracic or lumbar
epidural lesions would have missed secondary epidural lesions in only 1 percent of patients.
However, failure to image either the thoracic or lumbosacral spine missed lesions in 21 percent of
cases when symptomatic disease was located elsewhere.
Patients who cannot undergo MRI — Computed tomography (CT) myelography is the best
alternative to MRI in patients with suspected ESCC who cannot undergo MRI due to mechanical
valves, pacemakers, paramagnetic implants, embedded metal (eg, shrapnel), or severe
claustrophobia.
CT myelography involves the direct injection of contrast into the thecal sac by lumbar puncture,
followed by CT imaging of the spine. CT myelogram and MRI with contrast have roughly equivalent
sensitivity and specificity for the diagnosis of ESCC [3739].
Other situations in which CT myelography may retain utility include the following:
Occasional cases, particularly with laterally located lesions, in which CT myelogram
demonstrates abnormalities that are not visualized with MRI.
Myelography may be better tolerated by patients in considerable pain, since MRI depends upon
the ability of the patient to lie still.
Spinal instrumentation may cause significant artifact on MRI, preventing clear visualization of
the spinal cord in the region of spinal reconstruction. In such cases, myelography may be
necessary to visualize the spinal cord.
CT myelography is still widely used in treatment planning for radiosurgery.
Rarely, patients with complete subarachnoid block deteriorate neurologically when cerebrospinal
fluid (CSF) pressure below the block has been reduced by the lumbar puncture. For this reason, it is
frequently useful to have neurosurgical input when a CT myelogram is considered for suspected
ESCC.
Plain radiographics of the spine have no role in the diagnosis of ESCC. Although major vertebral
body collapse or pedicle erosion may indicate underlying ESCC in the proper clinical context, further
soft tissue imaging is always required, and falsenegative plain spinal radiographs occur in 10 to 17
percent of patients with ESCC [5,8].
Patients with prior spinal instrumentation — Spinal instrumentation may cause significant
artifact on MRI, preventing clear visualization of the spinal cord in the region of spinal
reconstruction. In such cases, CT myelography may be necessary to visualize the spinal cord.
Assessment of spinal stability — An important component of the decisionmaking process when
considering definitive therapy is assessment of spinal stability, which refers to the integrity of the
vertebral spine to resist progressive deformity and/or neural compromise under physiologic loads
[28]. Treatment of ESCC differs in those patients whose spine is unstable compared with those with
a stable spine.
Stability is best assessed by a spine surgeon based on radiographic features and clinical symptoms,
most importantly the presence or absence of movementrelated pain (table 1). Regardless of
radiographic findings, clinicians should consider every patient with pain caused by movement to be
unstable until proved otherwise. (See "Treatment and prognosis of neoplastic epidural spinal cord
compression", section on 'Mechanical (spinal stability)'.)
Cancer staging and role of diagnostic biopsy — In most patients presenting with ESCC who
have known metastatic cancer, biopsy of the compressive lesion is not necessary for treatment
planning, and a vertebral lesion with a typical appearance can be presumed to be metastatic. An
updated noninvasive cancer staging evaluation, typically by contrastenhanced CT of the chest,
abdomen, and pelvis or 18F fluorodeoxyglucose positron emission tomography (FDGPET)/CT,
aids in clinical decisionmaking in these patients. (See "Treatment and prognosis of neoplastic
epidural spinal cord compression", section on 'Baseline assessment'.)
The evaluation of patients with no, or a remote, history of cancer is more complex and must include
a prompt search for the primary site; contrastenhanced CT of the chest, abdomen, and pelvis
tailored for visceral organ evaluation plus a bone scan or FDGPET/CT may be used for
comprehensive staging evaluation. For patients in whom a likely primary tumor is identified, a
diagnostic biopsy of the primary tumor or the most accessible metastatic lesion should generally be
performed prior to definitive treatment of ESCC, in order to guide selection of the most appropriate
treatment modality.
In patients with highgrade ESCC, in whom surgical decompression and stabilization may be
indicated, multidisciplinary review with a radiologist and spine surgeon is indicated to determine
whether imageguided needle biopsy of the compressive vertebral lesion is appropriate prior to
surgery. For cases in which there is high suspicion radiographically for lymphoma or myeloma, a
core needle biopsy with expedited pathologic review may be appropriate, as confirmation of a
radiosensitive neoplasm would spare the patient an unnecessary decompressive spine surgery. For
patients with highgrade ESCC in whom the comprehensive staging evaluation is suspicious for a
radioresistant primary tumor such as renal cell or melanoma, diagnostic tissue can be obtained at
the time of surgical decompression and stabilization. (See "Treatment and prognosis of neoplastic
epidural spinal cord compression", section on 'Patients with unknown or synchronous primary'.)
DIFFERENTIAL DIAGNOSIS
Aside from ESCC, cancer patients are prone to a variety of other malignant and nonmalignant
causes of back pain and neurologic dysfunction.
Other causes of back pain in cancer patients — Benign or nonmalignant causes of back pain
that should be considered, especially in patients without evidence of ESCC on initial imaging of the
spine, include muscle spasm, intervertebral disk disease, and spinal stenosis. (See "Lumbar spinal
stenosis: Pathophysiology, clinical features, and diagnosis".)
One feature that is sometimes helpful in differentiating pain of ESCC from benign causes of back
pain is thoracic localization; the benign causes generally occur in the lumbar or cervical spine. The
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patient whose spine pain worsens while supine should not be presumed to have a benign cause.
Nocturnal pain and pain with neurologic deficit serve as "red flag" symptoms that warrant early
spinal magnetic resonance imaging (MRI), since neoplastic pain often worsens at night and pain
with neurologic deficit may signal neoplasm or infection.
Other neurologic complications of cancer — A number of other manifestations of metastatic
disease can cause severe back pain with or without neurologic deficits:
Vertebral metastases without epidural extension frequently produce local back pain, which
may be severe, especially in the presence of a pathologic compression fracture. Differentiation
from ESCC requires examination of adjacent soft tissue by MRI.
Intramedullary metastases occur much less frequently than epidural metastases and are most
commonly associated with lung cancer. They produce pain, weakness, and numbness similar to
ESCC. Unlike ESCC, they often produce a hemicord syndrome of unilateral weakness below
the level of the lesion with contralateral diminution of pinprick and temperature discrimination;
continued growth can lead to subsequent bilateral spinal cord dysfunction [40,41].
Intramedullary metastases may be missed on noncontrast imaging and are best visualized with
contrastenhanced spine MRI.
Leptomeningeal metastases can produce a cauda equina syndrome, which frequently
coexists with mental status changes, headache, and cranial nerve palsies [42,43]. Local back
pain may not be present, but affected patients usually complain of radicular leg pain. MRI
demonstrates no epidural tumor and may show pathologic thickening and nodular enhancement
of the cauda equina nerve roots and leptomeningeal enhancement along the surface of the
spinal cord. Cerebrospinal fluid (CSF) examination is usually diagnostic. (See "Clinical features
and diagnosis of leptomeningeal metastases from solid tumors", section on 'Diagnostic
evaluation'.)
Malignant plexopathy may involve the brachial or lumbosacral plexus. In most cases, the
major clinical feature of malignant plexopathy is severe unrelenting local or radicular pain. Later,
weakness and focal sensory disturbances occur in the distribution of the involved plexus [44]. In
the absence of a palpable mass or ipsilateral extremity swelling, these symptoms can mimic
ESCC. The paraspinal location of the plexus means that ESCC and malignant plexopathy often
coexist. MRI or computed tomography (CT) scanning can differentiate between these
possibilities. Brachial plexopathy most commonly occurs in patients with breast and lung
cancers. Lumbosacral plexopathy is usually due to colorectal and gynecologic tumors,
sarcomas, and lymphomas. (See "Brachial plexus syndromes", section on 'Neoplastic' and
"Lumbosacral plexus syndromes", section on 'Neoplastic invasion'.)
Radiation myelopathy should be considered in patients who have previously received
radiation to the spine or nearby structures. Rarely, patients may develop a chronic progressive
radiation myelopathy that shares clinical features with ESCC. The total previous radiation dose
and fraction size are important risk factors. The latency following radiotherapy is typically 9 to
15 months. (See "Complications of spinal cord irradiation".)
Affected patients usually develop painless ascending numbness and upper motor neuron
findings that often have a hemicord localization, presumably because radiation has produced a
vascular lesion [45,46]. The presence of a BrownSequard syndrome is helpful diagnostically.
MRI or myelography distinguishes this entity from ESCC.
Other causes of spinal cord compression — A number of other disorders can simulate some of
the findings in ESCC:
Spinal epidural abscess – Spinal epidural abscess is an uncommon but important condition to
recognize and distinguish from ESCC. Predisposing factors include intravenous drug use,
vertebral osteomyelitis, and hematogenous infection. The clinical manifestations may be
indistinguishable from rapidly progressive metastatic ESCC. Neuroimaging may suggest
infection but, in some cases, biopsy will be necessary for diagnosis. (See "Spinal epidural
abscess".)
The leading bacterial pathogen causing spinal epidural abscess is Staphylococcus aureus,
which accounts for approximately twothirds of cases of bacterial etiology. Other infectious
causes include Mycobacterium tuberculosis, which is responsible for up to 25 percent of cases.
(See "Skeletal tuberculosis".)
Vascular malformation – Vascular malformations of the spinal cord, such as dural
arteriovenous fistulas, can cause an acute or chronic progressive spinal cord syndrome with
local back pain or radiculopathy [47]. The diagnosis can be suggested by spine MRI with
contrast but generally requires magnetic resonance angiography and spinal angiography to
confirm.
Spinal epidural hematoma – Nontraumatic, spontaneous spinal epidural hematomas
occurring in the presence of anticoagulant therapy, arteriovenous malformations, or inherited or
acquired bleeding disorders are a rare cause of spinal compression [48]. The diagnosis can
usually be established by MRI [49].
Intradural extramedullary tumor – Meningiomas and neurofibromas can compress the spinal
cord and produce radicular and myelopathic syndromes. Their intradural location is usually
apparent on spine MRI with contrast.
Extramedullary hematopoiesis – Spinal cord compression can rarely be induced by
extramedullary hematopoiesis due to thalassemia or chronic myeloproliferative or
myelodysplastic disorders [50,51], as well as epidural involvement by rheumatoid arthritis,
sarcoidosis, or tophaceous gout [5254].
Degenerative spine disease – Intervertebral disc herniation and cervical or lumbar
spondylosis can cause acute or subacute myelopathy and radiculopathy, along with pain.
Spondylitic myelopathy is the most common cause of myelopathy in older adults. MRI of the
spine in the appropriate clinical context is diagnostic. (See "Cervical spondylotic myelopathy"
and "Lumbar spinal stenosis: Treatment and prognosis".)
SOCIETY GUIDELINE LINKS
Links to society and governmentsponsored guidelines from selected countries and regions around
the world are provided separately. (See "Society guideline links: Epidural spinal cord compression".)
INFORMATION FOR PATIENTS
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Basics topics (see "Patient education: Cauda equina syndrome (The Basics)").
SUMMARY
Neoplastic epidural spinal cord compression (ESCC) is a relatively common complication of
cancer that can cause pain and potentially irreversible loss of neurologic function. The most
common malignancies causing ESCC are prostate cancer, breast cancer, lung cancer, and
multiple myeloma. (See 'Epidemiology' above.)
The degree of thecal sac compression required for the designation of ESCC has been variably
defined. We use the term ESCC to mean any radiologic evidence of indentation of the thecal
sac, whether or not there are neurologic signs and symptoms associated with compression
(figure 1). (See 'Terminology' above.)
The most frequent presenting symptom of ESCC is back pain, which almost always precedes
neurologic dysfunction. Approximately 60 to 70 percent of cases occur in the thoracic spine, 20
to 30 percent in the lumbosacral spine, and 10 percent in the cervical spine. (See 'Clinical
presentation' above.)
Pain present only on movement frequently indicates mechanical instability of the spine. Spine
Instability Neoplastic Score (SINS) facilitates recognition of spinal instability and appropriate
referral for surgical evaluation (table 1). (See 'Pain' above.)
Motor findings represent advanced stages of ESCC. Unless there is profound weakness or
severe pain with movement, the motor examination in patients with ESCC must include
standing and walking in order to detect subtle weakness or ataxia. (See 'Motor findings' above.)
Urinary retention is the most common manifestation of autonomic dysfunction due to ESCC. It
is usually a late finding and is rarely the sole symptom. (See 'Bladder and bowel dysfunction'
above.)
Magnetic resonance imaging (MRI) with and without contrast provides the most accurate
evaluation of the thecal sac, adjacent soft tissues, and bone. The entire spine should be imaged
in patients with suspected ESCC, as clinical localization is imprecise and patients often have
multiple levels of involvement. (See 'Imaging features and grading' above and 'MRI of the entire
spine' above.)
Computed tomography (CT) myelography is the best alternative to MRI in patients with
suspected ESCC who cannot undergo MRI. (See 'Patients who cannot undergo MRI' above.)
In most patients presenting with ESCC who have known metastatic cancer, biopsy of the
compressive lesion is not necessary for treatment planning, and a vertebral lesion with a typical
appearance can be presumed to be metastatic. Select patients require imageguided needle
biopsy of the vertebral lesion for diagnosis and treatment planning. (See 'Cancer staging and
role of diagnostic biopsy' above.)
Aside from ESCC, cancer patients are prone to a variety of other malignant and nonmalignant
causes of back pain and neurologic dysfunction, all of which can be distinguished from ESCC
by history, examination, and MRI. (See 'Differential diagnosis' above.)
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