31
CLINICAL PEARLS
• Intramedullary spinal cord tumors (IMSCTs) are challenging
lesions that cause significant neurologic morbidity and
mortality in patients of all ages. Gross total resection should be
the mainstay of treatment, if possible, as survival is directly
related to extent of resection in this subset of patients.
• Careful evaluation of imaging to distinguish between the
various IMSCTs is essential, and if hemangioblastoma is a
possible diagnosis, preoperative vascular imaging is imperative
to provide guidance for navigation around the tumor so as to
minimize intraoperative blood loss.
Intradural Extramedullary Tumors
Dr. Horsely's attempted surgical resection of an intradural
extramedullary lesion that was producing progressive parapa-
resis in the late 19th century is one of the earliest reports of
spinal tumor surgery. 1 His successful resection of the fibro-
myxoma restored the patient's function and exemplified the
potential promise of intradural spinal surgery. A few decades
later, pioneers including Dr. Eiselsberg in Austria and Dr.
Elsberg in New York began to describe techniques to remove
intramedullary spinallesions. 2•3 By 1925 Elsberg was advocat-
ing a two-stage approach to spinal cord tumors involving
laminectomy and myelotomy followed by reexploration and
resection of tumor that had herniated out of the myelotomy. 4
Despite these initial successes, lack of technology and high
infection rates in the early 20th century hindered the rapid
development of intradural spinal surgery. With the advent of
microscopy, improved neuroimaging, and bipolar cautery,
however, intradural spinal tumor surgery had a resurgence in
the latter part of the 20th century, with results involving lower
morbidity and improved outcomes.
Epidemiology
Intradural tumors constitute a rare subset of tumors of the
spine because most spinal tumors arise from extradural or
500
• Intraoperative monitoring is an important and necessary
surgical aid to prevent postoperative neurologic deficits.
However, if patients who harbor a holocord lesion with or
without the presence of a syrinx lack transcranial motor evoked
potentials and reliable D-waves, great caution must be taken,
as these are the patients most prone to a poor neurologic
outcome after surgical intervention.
• Radiotherapy and chemotherapy have limited roles but are not
without their own treatment-related morbidities.
osseous structures. Three main types of tumors exist in the
spine: extradural extramedullary, intradural extramedullary,
and intradural intramedullary tumors. The latter two make up
a minority of spine tumors with an overall incidence of 1 and
2 per 100,000 persons. It is estimated that intradural tumors
account for 5% to 10% of central nervous system (CNS)
tumors.>-7 The most common intradural extramedullary
tumors include meningioma and schwannoma; however, the
differential diagnosis can be wide (Table 31.1).
Clinical Presentation
Most patients with intradural extramedullary masses present
with an insidious onset of symptoms that have gradually wors-
ened over time. Most of these symptoms are due to spinal cord
(meningioma) compression, nerve root (nerve sheath tumors)
compression, or a combination of both. The constellation of
symptoms may include sensory loss, nonspecific back pain,
ataxia, motor weakness, and lack of proprioception. Radicu-
lopathy followed by weakness may be more common in
patients with nerve sheath tumors. Weakness in the setting of
a nerve sheath tumor is a concerning sign for malignancy.
Cervical lesions may also present with headaches and occipital
pain due to involvement of the upper cervical roots. Clinicians
can frequendy miss thoracic tumors because they can present
with chest wall pain that is often mistaken for referred visceral
 CHAPTER 31 Intradural Extramedullary and Intramedullary Spinal Cord Tumors

• Differential Diagnosis for Intradural Extramedullary Tumors

Magnetic Resonance Imaging

Diagnosis Qualities Signal Characteristics Demographic
Nerve sheath tumors (schwannoma/ Enlargement of foramina T1 : lsointe nse Adults/pediatrics
neurofibroma/malignant peripheral Bony remodeling, with or without T2: Hyperintense
nerve sheath tumor) cystic features Enhancing
Meningioma Dural tail T1 : lsointe nse Mostly adults
lsointense T2: lsointense
Circumscribed Homogeneous e nhanceme nt
Paraganglioma Characteristic "salt and pepper" T1 : lsointe nse Mostly adults
appearance T2: Hyperintense
Conus/caudal location Avid enhancement
With or without associated
hemorrhage
Solitary fibrous tumors Well circumscribed T1 : lsointense Adults
T2: Hypointense
Avid enhancement
Myxopapillary ependymoma Cauda equina T1 : !so/hyperintense Adults/pediatrics
Conus location T2: Hyperintense
Expansile Enhancing
Dermoid/epidermoid Intradural well-defined mass T1 : Hypointense Pediatrics>
Mimic cerebrospinal fluid intensities T2: Hyperintense adults
Restricting on diffusion (epidermoid) Nonenhancing
Metastasis Variable T1 : lsointense Adults
Dural based T2: Hyperintense
Well circumscribed Enhancing
Neuroblastoma Heterogeneous lesion with necrotic T1 : Heterogeneous Pediatrics
or cystic areas T2: Hyperintense
Not well circumscribed Variable enhancement

ailments. Lesions of the cauda equina may produce a local mass
effect on lumbar roots that is alleviated when standing (less
engorgement of the epidural venous plexus). 8
A thorough examination of patients with intradural extra-
medullary lesions is essentiaL The tumor's spinal level should
correlate with the patient's signs and symptoms. Any incongru-
ence in the spinal level should be investigated thoroughly with
neuroimaging or nerve conduction studies to assess for addi-
tional lesions. A physical examination should also assess for
signs of myelopathy including Hoffinan signs, clonus, and
°
hyperrefiexia.9•1 For dorsal midline lesions, there may be an
indolent progression of dysesthesia to frank myelopathy. 11 •12
When the lesion is associated with a syrinx, it is not uncom-
mon to notice a suspended dissociated sensory level; however,
this may be more conspicuous in patients with intramedullary
lesions.s-'3
Imaging
Previously, the diagnosis of intradural extramedullary tumors
relied heavily on a detailed clinical examination and a com-
puted tomography (CD mydogram; however, with the advent
of magnetic resonance imaging (MRI), earlier tumor discovery,
localization, and tumor characterization have become feasible.
CT myelography is now more commonly reserved for patients
with contraindications to conventional MRI (eg, those who
have pacemakers or foreign ferromagnetic objects). 14
In the assessment of spinal tumors, contrast MRI permits
identification of the lesion and its proximity to adjacent vas-
culature. Additionally, a lack of contrast enhancement may
portend a less ominous diagnosis such as spinal lipomas or
arachnoid cysts. For patients with long-standing symptoms or
patients with previous tumor resections, plain radiographs can
be helpful for assessing sagittal and coronal imbalance or
potential instability. Table 31.1 includes an overview of typical
characteristic MRI imaging of intradural extramedullary
tumors.
In cases with multiple intradural lesions or systemic disease,
the use of positron emission tomography (PET/CT) may help
to define the degree of metabolic activity within the tumor.
Metastatic lesions to the dura such as lymphomas, renal cell
carcinomas, or sarcomas may be hypermetabolic on PET/CT
imaging; however, the resolution ofPET/CT imaging is much
poorer than that of conventional MRI! >-17
502 PART 5 The Spine
• Figure 31.1 A 7-year-old boy with symptoms of myelopathy and left arm weakness. Cervical spine
computed tomography (A} and magnetic resonance imaging (B} demonstrating an intradural calcified
mass with canal compression suggestive of meningioma.
Surgery
The mainstay surgical approach for intradural extramedullary
lesions is a posterior midline laminectomy that can be manipu-
lated depending on the location of the lesion. Once fluoro-
scopic localization of the lesion is confirmed, laminectomies
generally can be performed without disrupting the facet
complex. Lateral extraspinal exposures (thoracotomy, costo-
transversectomy) are also helpful in some anterolateral-based
thoracic lesions. 18 In children, laminoplasties can be performed
to maintain the integrity of the posterior columns. Once the
bony exposure is complete, adjuvant imaging modalities such
as ultrasound can help guide the dural opening. 19 1he dura can
be opened widely and tacked up to the adjacent musculature.
Depending on the adherence and tumor interface, an attempt
at en bloc resection can be made. If the dissection planes are
poor, piecemeal resection with bipolar cautery or ultrasonic
cavitation can be performed. Maximal safe resection is always
the goal of intradural tumor surgery; however, in some cases,
sacrifice of a sensory nerve root is necessary for complete
removal. Because of the risk of severe neurologic sequelae,
intraoperative monitoring and appropriate preoperative coun-
seling are crucial.
Spinal Meningiomas
Meningiomas are the most common type of intradural extra-
medullary tumors, and 80% of all cases affect women. Patients
classically present with myelopathy or sensory disturbances.20.2 1
CT imaging of meningiomas may demonstrate some degree of
calcifications, and most meningiomas homogeneously enhance
(Fig. 31.1). Thoracic (70%-75%) and cervical (20%) menin-
giomas are the most common locations for intraspinal menin-
giomas.s,.22 Although the pathophysiology is unclear, some
authors have suggested that meningiomas arise from dural
fibroblasts in the arachnoid layer.23 At times, meningiomas can
be highly vascular; therefore careful evaluation of the preopera-
tive imaging and adjacent vasculature is essential. In the spine,
histology is typically ben~, and gross total resection is the
mainstay of treatment.22' With gross total resection, recur-
rence rates can remain quite low. Close follow-up is recom-
mended with serial imaging if there is concern for a high Ki-67
mitotic index or atypical histology.
Surgical Approach
Given the histology, typically meningiomas can be removed by
a combination of lesional cautery, debulking, and resection of
the dural attachment. Meningiomas can frequendy be calci-
fied, which may complicate surgical excision; in these cases,
ultrasonic cavitation may be helpful. If the tumor is densely
adherent to adjacent neural elements, complete resection
should be avoided. Evidence suggests that subtotal resection or
coagulation of the dural attachment (Simpson grade II) should
be considered to avoid neurologic injury in patients with
difficult-to-treat spinal meningiomas. 2>-27 A complete tumor
removal would necessitate excision of the dural attachment,
which would demand a dural patch. Duraplasty with cadaveric
 CHAPTER 31 Intradural Extramedullary and Intramedullary Spinal Cord Tumors
dura and fibrin glue can be used to repair the dural defect in
a watertight fashion in these cases.
Nerve Sheath Tumors
The second most common intradural tumor is the nerve sheath
tumor, which can account for 30% of spinal tumors.28 The
nerve root sleeve tends to be enlarged as these tumors arise
from the nerve root. This group of tumors tends to originate
from Schwann cell or nerve root fibers. We will discuss both
common histologic subtypes (schwannomas and neurofibro~
mas) separately.
Schwan nomas
Schwannomas are the most common nerve sheath tumor,
accounting for nearly 85% of all neoplasms in this location. 29
They tend to be well~demarcated, contrast~enhancing masses
with possible cystic components. Additionally, there may be a
characteristic dumbbell shape as the nerve exits the neural
foramen. Because the tumor displaces nerve fibers, the goal of
surgery is gross total resection with sparing of the nerve fibers.
Intraoperative monitoring with somatosensory evoked paten~
rials and motor evoked potentials may help guide resection in
cases where there is difficulty distinguishing neural and tumoral
elements. If multiple schwannomas are seen on imaging, pas~
sible diagnoses of neurofibromatosis or schwannomatosis
should be investigated.30
Neurofibromas
The second most common nerve sheath tumors, neurofibro~
mas, account for 15% to 20% of all nerve sheath tumors and
occur mostly in patients with neurofibromatosis (NFI). Fre~
quently; patients with syndromic neurofibromas harbor mul-
tiple lesions that may demonstrate malignant/aggressive
histologic features. 31 Like schwannomas, these tumors tend to
arise from the sensory roor2 9•32; however, unlike schwannomas,
neurofibromas displace nerve fibers radially because the tumors
arise intrinsically. Careful preoperative discussions with the
patient and family about the risks of postoperative weakness
and goals of surgery are necessary.
Surgery
As with any intradural tumor, surgery for nerve sheath tumors
is specific to the tumor's location and the characteristics
observed on preoperative imaging. Posterior/posterolateral
approaches can be utilized to access most dorsal/dorsolateral
tumors. For lesions that are intimately involved with nerve
roots, aggressive surgical resection of these lesions is often not
possible without sacrificing the nerve root. Like meningiomas,
nerve sheath tumors are amenable to ultrasonic aspiration/
cavitation. In this group of tumors, neurofibromas tend to be
the most difficult to remove because of the radial displacement
of nerves and the lack of a dissection plane. Many times,
patients with poor preoperative nerve function can tolerate
sacrifice of the nerve root without a significant change in their
postoperative neurologic status.33•34
Paragangliomas
Paragangliomas arise from sympathetic nerve cells and are typi-
cally found in the cauda equinalfilum terminale regions in the
spine. Because of their vascularity, they are contrast~nhancing
lesions with a relatively well-circumscribed tumor-neural inter-
face. As with intracranial paragangliomas, spinal paraganglia~
mas possess a stereotypical histology with dusters of Zellballen
and chief cells; however, it must be noted that spinal paragan-
gliomas naturally do not produce sympathetic symptoms. If
possible, aggressive surgical resection is warranted with a
potential for adjuvant radiotherapy for residual or recurrent
masses.
Dermoid/Epidermoid Lesions
Although most commonly found intracranially, dermoid/
epidermoid cysts can occur in variety of locations in the spine
(intradural, intramedullary, extradural, etc.). Congenital
tumors arise from ectopic embryonal ectoderm as a result of
defective cell disjunction. 35 It is also possible to develop
acquired epidermoid/dermoid tumors after surgical procedures
and spinal punctures that introduce cutaneous tissue into the
spinal canal. MRI demonstrates lesions that possess similar
intensities to cerebrospinal fluid with restricted diffusion. In
the operating room, epidermoid lesions have been described as
a pearly white tumor (tumor perlee). Thorough surgical plan-
ning will help to avoid and mitigate risks of aseptic meningitis
(Mollaret meningitis) associated with cyst rupture.
Lipomas
Intradural lipomas typically occur in the lumbar spine and
usually afHict children with occult spinal dysraphism. Almost
uniformly, spinal lipomas are benign lesions that cause a local
mass effect on nerve roots. As a result, surgical resection and
decompression may be necessary. Additionally, any associated
dermal tracts or fistulas should also be evaluated and removed.
Intradural Lesions That Mimic Tumors
Several lesions can mimic tumors in the intradural space. Table
31.2 summarizes some of the radiographic features of these
lesions.
Arachnoid Cysts
Arachnoid cysts in the spine are non~neoplastic collections of
cerebrospinal fluid under the arachnoid layer that are mostly
asymptomatic. Rarely, arachnoid cysts can grow and produce
mass effect on the spinal cord, producing myelopathy or nerve
root compression. Because of the narrow canal diameter,
arachnoid cysts in the thoracic spine seem to be the most
symptomatic. Treatment for these lesions may include
504 PART 5 The Spine
• Non-Neoplastic Intradural Extramedullary Lesions
Diagnosis Magnetic Resonance Imaging Qualities
Lipoma Children: cervical spine
Adults: thoracic spine
Dorsal midline lesion, similar to fat
Intradural disk Associated with disk space, cord
herniation compression
Arachnoid cyst Cerebrospinal fluid-like mass
Rare mass effect
Circumscribed
Dural arteriovenous Flow voids
malformations With or without cord signal change
Enlarged perimedullary veins
Sarcoidosis Circumscribed mass Ontramedullary or
extramedullary)
With or without dural tail sign
anything from a simple fenestration to placement of a sub-
arachnoid shunt for refractory lesions. 36
Synovial Cysts
Synovial cysts are commonly found extradurally in the lumbar
spine and produce symptomatic radiculopathy and back pain.
Repetitive motion and degenerative processes along the facet
capsule can exacerbate synovial cysts and worsen symptomatol-
ogy. At times, these lesions can be mistaken for intradural
nonenhancing lesions; therefore a careful preoperative workup
is necessary to distinguish synovial cysts from a more ominous
diagnosis. The symptomatology; facet location, and lack of
enhancement can help facilitate prompt and accurate diagno-
sis. Management of these lesions is typically conservative but
may include percutaneous aspiration or lumbar decompression
and fusion. 37•38
Adjuvant Treatments
In some cases, adjuvant treatments may be necessary for
local control of refractory lesions. Radiation and chemo-
therapy are the typical mainstay adjuvant treatment options
for these lesions. It is important to know that radiation is
usually reserved for patients with large residual tumors with
an atypical/malignant histology or recurrent extramedullary
tumors. For malignant lesions with grade IIIIN histology,
adjuvant radiation can be commenced rapidly after surgery.
For less aggressive tumors (atypical meningiomas, or schwan-
nomas), radiation can be reserved for tumor recurrence.39
Given the proximity to the spinal cord, intradural lesions can
be difficult to irradiate. As such, radiosurgery has been effective
at delivering focused radiation to the tumor bed and sparing
neighboring neural dements. Additionally, radiosurgery can be
utilized in some settings as a primary treatment for patients
who are poor surgical candidates.40 Care must be taken to
Signal Characteristics Demographic
T1 : Hyperintense Pediatrics> adults
T2: Hypointense
Nonenhancing
Similar intensity as associated disk Adults
Incomplete enhancement
T1 : Hypointense Adults/pediatrics
T2: Hyperintense
Nonenhancing
T1 : Hyperintense Adults
T2: Hypointense +enhancement
T1 : Heterogeneous Adults
T2: Heterogeneous
Enhancing
ensure that the adjacent spinal cord receives no more than
10 Gy of radiation. Radiation-induced spinal toxicity has been
reported in a delayed fashion (>6 months) in patients who
have received spinal radiation. As a result, radiation has had a
relatively limited role for intradural lesions with surgery as the
mainstay of treatment.41 '40
Other adjuvant modalities have also been suggested, includ-
ing MR-focused ultrasound (MRFUS) and laser interstitial
thermal therapy (LITI). Although both are mostly applicable
to intracranial lesions, MRFUS and LITI have theoretic appli-
cability to some treatment-refractory or surgically inaccessible
intradural extramedullary lesions. MRFUS utilizes acoustic
waves to generate coagulative thermal energy (60°C) in a rela-
tively small, focused field that can be monitored with MRI.
For nonablative MRFUS, less energy (ie, lower temperatures)
is generated over a longer interval to generate internal cavita-
tion and microshearing of tissues. Other potential effects
of MRFUS include blood-brain barrier disruption and
immunomodulation. 42•43
Similar to MRFUS, LITI stimulates thermal energy to
induce tissue destruction that can be monitored with MRI.
However, LITI requires a catheter to be introduced and sta-
bilized within the lesion. For intracranial intradural lesions,
LITI has been tested with some success and minimal morbid-
ity; however, for spinal intradural lesions, catheter placement
and stabilization may be difficult.44.45 Nevertheless, LITI treat-
ment may be a potential option for extradural bony metastasis
and potential malignant intradural lesions in the near future
as catheter-based technology improves.
Intramedullary Spinal Cord Tumors
Intramedullary spinal cord tumors (IMSCTs) are rare lesions
that account for 2% to 4% of all CNS tumors.46 1hey account
for 20% of all intraspinal tumors in adults and 35% of all
intraspinal tumors in children. 47 1he pathology differs between
 CHAPTER 31 Intradural Extramedullary and Intramedullary Spinal Cord Tumors

the pediatric and the adult population, with ependymoma
being the most common entity in the adult population and
astrocytomas being the most common in children and adoles-
cents. Overall, ependymomas are the most frequent IMSCTs,
fOllowed by astrocytomas and then other miscellaneous tumors.
Other tumors that can be found within the intramedullary
spinal cord include hemangioblastomas, gangliogliomas, para-
gangliomas, primary CNS lymphomas, mdanomas, and epi-
dermoid cysts (Table 31.3). Occasionally, IMSCTs can develop
as a result of metastasis from a primary malignancy. IMSCTs
represent a significant clinical challenge mainly because of the
lack of a dear standard of care, limited therapeutic options
given the doquent location of these lesions, and challenges
associated with drug delivery.
IMSCTs can be round in any location throughout the
length of the spinal cord. These lesions are most commonly
round in the cervical spinal cord (33%), followed by the tho-
racic spinal cord (26%), with the least likely location being in
the lumbar spinal cord at the levd of the conus (24%).48 It has
been proposed that the higher propensity of IMSCTs for the
cervical spine is directly related to the higher percentage of gray
matter present at that level.48 Magnetic resonance imaging is
the gold standard imaging modality for evaluating IMSCTs;
varying intensities and contrast enhancement are used depend-
ing on the tumor type.
The most common presenting symptom of IMSCfs is
diffuse or radicular back pain. The pain can be variable but
can be worse at night. Diagnosis in children can often be
challenging because IMSCTs can cause nonspecific com-
plaints.49 Progressive scoliosis is present in 33% of children
with IMSCTs, with or without the presence of back pain as
the initial presenting symptom. Motor regression and frequent
falls may be the presenting symptom in very young children,
and this is often mistaken for clumsiness, which can dday
diagnosis. 49 IMSCTs can also impinge on the spinal, somato-
sensory, and motor tracts, leading to paresthesias or dysesthe-
sias, loss of dorsal columns with loss of vibratory and position
sense, spasticity; and weakness. Loss of bladder and bowel
function tends to be the least common presenting symptom
of IMSCTs and typically occurs at the later stages of diag-
nosis after the aforementioned symptoms have already come
to attention.49' 50
Typical treatment paradigms for IMSCTs primarily involve
resection or biopsy. Adjuvant therapy including chemotherapy
and radiation is generally reserved for tumor recurrence or
high-grade pathology in which there was incomplete resection
because of concern for neurologic injury. These treatment para-
digms are based on class I and II evidence. The best predictors
of outcome are preoperative neurologic status and tumor his-
tology.51 Tumor histology has been shown to predict the extent
of resection that can be achieved as well as functional neuro-
logic outcomes and rate of recurrence. Gross total resection
(GTR) is typically considered a good predictor of outcome,
but evidence suggests that surgical management of astrocytoma

• Differential Diagnosis for Intramedullary Spinal Cord Tumors

Magnetic Resonance Imaging

Diagnosis Qualities Signal Characteristics Demographic
Ependymoma Heterogeneous expansile mass T1 : lso- to hypointense Adults > pediatrics
lntratumoral or peripheral cysts T2: Hyperintense
Hemorrhagic foci common in or at Intense homogeneous enhancement
margins of tumor (cap sign)
Myxopapillary Vertebral remodeling changes T1 : lsointense Adults > pediatrics
ependymoma Eroded pedicles T2: Hyperintense to cord
Intervertebral foramina! widening Intense enhancement
Astrocytoma Oblong, fusiform expansion of the T1 : lsointense to hypointense Pediatrics > adults
spinal cord T2: Hyperintense to normal cord
Rarely hemorrhagic Majority enhance moderately
Hemangioblastoma Dorsal surface of the spinal cord with T1 : Nodule hypointense with brain Adults > pediatrics
prominent flow voids and presence of +1- flow voids
a nodule T2: Both nodule and cyst are
hyperintense to brain
Nodule avidly enhances
Ganglioglioma Holospinal involvement T1 : Mixed signal intensity Pediatrics > adults
Absence of edema T2: Homogeneous > heterogeneous
Patchy enhancement with no Patchy enhancement
enhancement within the center of the
tumor
Metastases Focal enhancing lesion(s) with extensive T1 : Hypolisolhyperintense Mainly adults
edema T2: Hyperintense
Focal enhancement, rim sign
506 PART 5 The Spine
is associated with higher rates oflong-term neurologic compli-
cations with no derived benefit for the patientY
The decision to perform a biopsy or resection is soldy
dependent on the presence or absence of a dear plane of dis-
section between normal spinal parenchyma and tumor, 53 which
varies among pathologic subtypes of IMSCTs. Ependymomas
typically have a dear plane between the tumor and spinal cord
parenchyma, whereas astrocytomas are notorious for their in6.1-
trative properties, which blur the limits of normal spinal cord
parenchyma and tumor margins. This becomes the biggest
limiting factor in achieving a GTR for intramedullary astrocy-
tomas and leads to a higher risk of damaging the ascending
and descending spinal cord tracts during surgery, leading to
neurologic deficits. 53
Intraoperative neurophysiologic monitoring (IOM) is a
useful surgical tool that can be used during biopsy or resection
to hdp avert worsened neurologic function after surgical inter-
vention. Both transcranial motor evoked potentials (MEPs)
and sensory evoked potentials (SEPs) are important for assess-
ing the integrity of the spinal cord as resection ensues. The
combined used of SEPs and MEPs in intramedullary spinal
cord tumor surgery is almost mandatory because of the pos-
sibility of sdectively injuring either the somatosensory or the
motor pathways. Besides standard conical SEP monitoring
after peripheral stimulation, both muscle (mMEPs) and epi-
dural (D-wave) MEPs are monitored after transcranial electri-
cal stimulation (TES). During tumor removal, the surgeon
should alternativdy monitor D-wave and mMEPs, sustaining
the stimulation during the most critical steps of the procedure.
D-waves, obtained through a single-pulse TES technique,
allow a semiquantitative assessment of the functional integrity
of the corticospinal tracts and are the strongest predictor of
motor outcome after surgery. If potentials are lost, the proce-
dure should be halted so the wound can be irrigated copiously
with warm saline, the blood pressure increased, and anesthetic
use can be assessed until the potentials recover. 54 If potentials
do not recover, aborting the procedure and waking up the
patient is the best course of action. In patients who harbor a
holocord lesion with or without the presence of a syrinx, great
caution must be taken because they lack transcranial MEPs
and reliable D-waves and are the most prone to a poor neuro-
logic outcome after surgical intervention. In this subset of
patients, biopsy, rather than resection, should be the goal, as
the risks to the patient are significant.
The role of radiotherapy in patients with IMSCfs is unde-
cided, with some reports of a favorable outcome and other
reports showing no benefit despite its routine use within a
clinical setting. Adjuvant radiotherapy can be utilized when
GTR is contraindicated because of concern for neurologic
injury or in patients harboring high-grade pathology. The use
of adjuvant radiotherapy is not without its risks, as there
are associated adverse effects including radiation myelopathy,
impairment of spinal growth, spinal deformities, radiation
necrosis, vasculopathy, restrictive lung disease, changes to
the normal spine parenchyma, and a risk of secondary malig-
nancies seen in 25% of patients within 20 years.46•55•56 These
long-term adverse effects are particularly profound in
the pediatric population, and parents must be appropriately
counsded.49
Chemotherapy is controversial for the treatment ofiMSCfs,
but it is more accepted. in the pediatric population because of
the patients' increased propensity toward adverse effects from
radiation.49 Chemotherapy was historically used. only when
resection and adjuvant radiotherapy were unsuccessful or con-
traindicated, but chemotherapy has its own limitations, specifi-
cally its inability to penetrate the blood-spinal cord barrier
(BSCB), cerebrospinal fluid pulsations and systemic effects of
the chemotherapeutic drugs. Chemotherapy should not be
considered a benign alternative to radiotherapy. For example,
Allen and colleagues57 reported that 75% of their pediatric
patients with high-grade IMSCfs experienced significant
chemotherapy-associated toxicity, with one death related to
acute renal failure and another patient with bilateral hearing
loss. With the current treatment modalities, the lack of positive
outcomes, and the localized nature of IMSCfs, we are in des-
perate need of novel therapeutic modalities directed at molecu-
lar pathways with targeted drug ddivery and localized drug
delivery mechanisms capable of bypassing the BSCB to avoid
the toxic systemic side effects.
Ependymomas
Ependymomas are the most common IMSCfs in adults (par-
ticularly males), accounting for 50% to 60% of all intramedul-
lary tumors in this population, and the second most common
IMSCfs in the pediatric population.49•58 Ependymomas arise
from the ependymal cells lining the ventricles of the brain and
the central canal of the spinal cord. Ependymomas are classi-
fied into four distinct histologic subtypes: cellular, papillary,
clear cell, and tanycytic.47 With few exceptions, these tumors
are histologically benign, although they exhibit some biologic
variability with respect to growth rate. Most are World Health
Organization (WHO) grade II, but myxopapillary ependymo-
mas are WHO grade I lesions and anaplastic ependymomas
are WHO grade III lesions. Myxopapillary ependymomas
account for >50% of cases. They generally arise from the filum
terminale of the spinal cord and are usually located in the
cauda equina of the spinal cord. 59•60 The other three subtypes
of ependymoma have the typical distribution of IMSCfs and
are usually found in the cervical and thoracic spinal cord. The
majority of ependymomas are slow-growing lesions and present
with chronic back pain,59 but anaplastic ependymomas tend
to be more aggressive and have a more acute presentation.
On imaging, ependymomas are heterogeneous expansile
masses in 50% to 90% of patients; they display intratumoral
or peripheral cysts with or without the presence of syrinx.
Hemorrhagic foci are common in or at the margins of the
tumor. Typically, ependymomas are multisegmental, spanning
on average three to four spinal levds. They tend to be
hyperintense on Tl-weighted imaging and hypointense on
T2-weighted imaging. They have avid heterogeneous enhance-
ment with contrast administration (Fig. 31.2). They tend to
have an affinity for the cervical spine followed by the thoracic
spine and then the conus. Myxopapillary ependymomas are
 CHAPTER 31 Intradural Extramedullary and Intramedullary Spinal Cord Tumors
• Figure 31.2 A 22-year-old male with symptoms of lower extremity radiculopathy and bladder incon-
tinence. MRI T1 with contrast (A) and T2 (8) showing heterogeneous enhancing lesions in entire cauda
equina suggestive of ependymoma.
classically located at the conus as previously stated but can also
occur in the soft tissues of the sacrococcygeal region.59•60
Ependymomas are unencapsulated, noninfiltrative IMSCfs
and generally present a clear margin of demarcation from the
surrounding spinal cord that serves as an effective dissection
plane. For this reason, GTR is the primary form of treatment
for patients harboring these lesions. Guidetti and colleagues61
have shown that the extent of resection is a strong predictor of
overall survival, with >90% of patients showing improvement
in symptoms following GTR. For patients with myxopapillary
ependymomas, GTR alone is associated with decreased recur~
renee rates as compared with subtotal resection (STR) with or
without radiotherapy. The treatment goals should always
include attempted GTR whenever possible. Feldman and col~
leagues62 also observed that children with myxopapillary epen~
dymomas benefited from radiation therapy to a greater extent
than did adults, suggesting that inherent biologic differences
exist between tumors of the pediatric and adult populations,
warranting more rigorous scientific studies.
Adjuvant radiotherapy is not indicated in the setting of
GTR but is used in the setting of STR, recurrent tumors, and
lesions that cannot be surgically accessed. Feldman and col~
leagues62 also reported that radiotherapy was not associated
with lower overall recurrence regardless of the extent of resec~
tion. Further prospective studies looking at the benefit and risk
profile of radiotherapy in this subset of patients are needed to
develop a clear delineation of the risk~benefit profile of radio~
therapy in these patients.
The use of chemotherapy with ependymoma is controver~
sial, and good data are lacking; however, its utility within the
pediatric population is important because this population has
the most morbidity associated with adjuvant radiotherapy.
Charnberlain63•64 showed that there is a modest response to
etoposide (a topoisomerase~2 inhibitor) with a partial response
in 20% of patients treated. The study was limited by the small
number of patients within the study group but highlights the
importance at looking at novel chemotherapeutic agents.
Astrocytomas
Astrocytomas are the most common IMSCTs in children, rep~
resenting over 60% of all IMSCTs in this population, and the
second most common IMSCfs in adults. 65 Astrocytoma is a
primary intramedullary neoplasm of the spinal cord originat~
ing from astrocytes. The majority are low~grade lesions such as
juvenile pilocytic astrocytomas QPAs) WHO grade I, with a
smaller subset of patients harboring fibrillary astrocytomas
WHO grade II lesions and anaplastic astrocytomas.65 These
typically come to presentation in preadolescents between 5 and
10 years of age with symptoms of scoliosis, pain specifically
worse at night, myelopathy, abdominal pain, and muscle
wasting. 47,49,65
On imaging, astrocytomas tend to be enhancing infiltrating
spinal cord masses. They have a predilection for the cervical
spine followed by the thoracic and then the lumbar spine. They
tend to be 1 to 3 em in length and generally span fewer than
4 spinal segments but can also have holocord lesions that are
present. They tend to be oblong with fusiform expansion of
the cord. Forty percent have tumor cysts or syringohydromy~
elia with the cyst fluid slighdy hyperintense to cerebrospinal
fluid. The solid portion of the tumor is hypo~ to isointense
on Tl~weighted imaging and hyperintense on T2-weighted
508 PART 5 The Spine
imaging. These lesions are rarely hemorrhagic and have mild to
moderate enhancement with contrast administration. Usually,
it is challenging to distinguish between astrocytomas and epen-
dymomas on magnetic resonance imaging (MRI) appearance
alone but astrocytomas are occasionally asymmetrical or off
center, which distinguishes them from ependymomas.59
Astrocytomas are more infiltrative lesions than ependymo-
mas and typically lack a clearly defined cleavage plane between
normal spinal cord and the lesion itsd£ For this reason, GTR
is usually not achieved without severe neurologic deficits, and,
as a result, patients harboring these lesions have a worse overall
prognosis. Raco and colleagues50 demonstrated that only 12%
of patients with WHO grade II lesions underwent GTR, and
no patients harboring WHO grade III or IV lesions were able
to achieve a GTR. Babu and colleagues52 showed that 37% of
patients in their series of46 patients undergoing surgical resec-
tion for astrocytomas had worse postoperative neurologic defi-
cits than preoperatively, underscoring the infiltrative nature of
these tumors and the difficulty in discerning tumor from
normal spinal cord parenchyma. For this reason, GTR is not
recommended and instead STR with preservation of neuro-
logic function is of utmost importance. However, as noted by
Karikari and colleagues, 51 47.6% of patients with spinal cord
ependymomas undergoing STR will have a recurrence. This is
in stark contrast to 7.3% of patients with recurrent ependy-
moma after STR51
If there is a recurrence of astrocytoma, radiotherapy is the
standard treatment for these patients.46 Given that the major-
ity of patients with astrocytomas are children, the risk profile
of radiotherapy can be problematic. There is limited evidence
that supports chemotherapy for the use of astrocytomas. There
have been limited studies that show the effectiveness of temo-
zolomide as a possible adjuvant treatment in these patients.
Chamberlain and Tredway46 showed that temozolomide treat-
ment led to 27% progression-free survival at 2 years with
a median survival of 3 months in patients with recurrent
astrocytoma; however, there were also treatment-associated
toxicities, which underscores the importance of developing
novel therapeutic agents that can directly target the specific
levels of the spinal cord that are affected to avert the systemic
toxic effects.
Hemangioblastomas
Intramedullary hemangioblastomas account for 3% to 4% of
all IMSCTs and are the third most common IMSCT in adults
after ependymomas and astrocytomas.66 1hey derive from mes-
enchymal cells that originate from the vascular system within
the spinal cord and cerebellum. As a result of the close relation-
ship with the vascular system, these lesions are capillary rich
and tend to possess a high degree of vascularity and angiogen-
esis with growth. They are most commonly encountered within
the cerebellum (83%), but 13% are found within the spinal
cord. 67
Approximately 10% to 20% of patients who are diagnosed
with spinal cord hemangioblastoma have von Hippel-Lindau
(VHL) disease.46 It is important to do a comprehensive workup
in any patient who presents with an intramedullary hemangio-
blastoma to rule out the presence ofVHL disease and any other
possible lesions such as pheochromocytomas, pancreatic cysts,
multiple hemangioblastomas, and renal cell carcinoma. In
patients with VHL, the gene that is mutated leads to enhanced
transcription of several genes including vascular endothelial
growth factor (VEGF), which likely contributes to the devel-
opment of vascular tumors such as hemangioblastomas. 47
Hemangioblastomas have a propensity for the dorsal portion
of the spinal cord. As a result, patients typically present with
dorsal column dysfunction with progressive sensory and pro-
prioceptive deficits. Patients can also present with hemorrhage,
either subarachnoid (73%) or intramedullary (27%), related
to the vascularity of the lesions and an acute neurologic
deterioration. 68
Hemangioblastomas have a propensity for the thoracic
spinal levels followed by the cervical, lumbar, and sacral levels.
They tend to be located subpially in the dorsal aspect of the
spinal cord, and they demonstrate uniform contrast enhance-
ment on imaging. They often have vessel flow voids that are
apparent on different MRI sequences and are often associ-
ated with intraspinal cysts. An extensive syrinx apparent on
imaging is also highly suggestive of the presence of a heman-
gioblastoma. Because of the alterations in the vasculature of
the spinal cord, there may be diffuse spinal cord thickening
remote from the tumor (without the presence of a syrinx) due
to edema. 58 Because of the hypervascularity, hemangioblasto-
mas are almost always differentiated from ependymomas and
astrocytomas. 67~9
Hemangioblastomas tend to be WHO grade I lesions and
typically have no associated malignant degeneration. Resection
is the primary treatment for intramedullary spinal cord heman-
gioblastomas, as they tend to have a clear dissection plane that
makes them amenable to GTR It is important to obtain
vascular imaging of the lesions prior to surgical resection to
identify the dominant feeding vessel as well as the draining
vein exiting. Depending on the experience level of the surgeon
and the interventionalist, embolization can be considered if
feasible to make the surgical extirpation of the lesion easier.
The strategy of microsurgical removal of the tumor is to coagu-
late the dominant arterial feeders at the entrance of the tumor
by bipolar coagulation with low power first, then to coagulate
and shrink the tumor, and finally to coagulate the venous
drainage prior to removing the lesion en bloc. 58
Because GTR is usually achieved with intramedullary
hemangioblastomas, radiotherapy and chemotherapy have not
been extensively researched with this pathologic subtype. The
limited studies of antiangiogenesis therapies have had mixed
results, implying that some hemangioblastomas might show
responsiveness to angiogenesis inhibitors whereas others may
not, depending on the level of up-regulation of the VEGF
gene.69,70
Gangliogliomas
Gangliogliomas are rare IMSCTs that arise from both neuronal
and glial origins and are composed of both glial and ganglion
 CHAPTER 31 Intradural Extramedullary and Intramedullary Spinal Cord Tumors
cells. 71 These lesions tend to present in the pediatric popula-
tion, with the highest incidence in the first three decades. In
a series of 348 patients with ganglioglioma, older children and
young teenagers were the most likely affected.72 These lesions
tend to he found in the cervical spinal levels and tend to halo-
spinal involvement. They are difficult to distinguish on MRI,
where there may he patchy enhancement or no enhancement
within the center of the tumor. There may also he hemosiderin
or calcification, and there tends to he an absence of edema.
Scoliosis with bony remodeling is a common finding among
patients with this pathology. Patients also tend to present with
hack pain, limb weakness, and progressive myelopathy. 47•73•74
These are typically benign lesions, either WHO grade I or
II, although malignant degeneration has been reponed and
tends to involve the glial component of the tumor. For this
reason, as with other IMSCfs, biopsy and resection are the
primary treatment. GTR is attained at a much higher rate
(83.3%) in spinal gangliomas than in their intracranial coun-
terparts72; however, because of the location of these tumors
within the cervical spine and the infiltrative nature of the glial
component, GTR needs to he weighed against its risk of wors-
ening postoperative neurologic function. The roles of radio-
therapy and chemotherapy are not well understood within this
pathologic subtype, and further studies need to he done to
better understand the efficacy of these modalities. Spinal gan-
gliogliomas have a higher relative risk of recurrence than both
cerebral and hrainstem gangliogliomas and have a 10-year sur-
vival of 83%.66•73
Intramedullary Metastases
Intramedullary metastases are rare lesions, affecting 0.4% of
patients with malignancies, and represent 1% to 3% of all
IMSCfs. 55 The primary tumors that are most prone to intra-
medullary metastases are lung, breast, and lymphoma.47·55 The
prognosis for this subset of patients is dismal, and for this
reason, fast and accurate diagnosis is imperative for survival.
Hashii and colleagues75 showed that the median survival in this
population of patients is less than 4 months, and none of their
patients achieved remission regardless of what treatment was
offered. Because there is a complete lack of a dear plane
between the metastatic lesion and the normal spinal cord
parenchyma coupled with the dismal prognosis, surgical inter-
vention does not seem to he a reasonable option. Quality of
life should be the main factor that is stressed in this subset of
patients. Radiotherapy has been used with some benefit with
regard to the lesion but with deleterious side effects to the
surrounding normal spinal cord parenchyma and the expected
worsening of neurologic function. Only isolated case reports
in the literature describe any benefit to using chemotherapeutic
agents in this subset of patients. 75•76
Selected Key References
Bruneau M, George B. Surgical technique for the resection of tumors in
relation with the V1 and V2 segments of the vertebral artery. In:
George B, Bruneau M, Spetzler RF, eds. Pathowgy and Surgtry Around
tM VmebralArtoy. Paris: Springer; 2010. Chap. 14.
Fisher G, Brotchi J. Intramedullary Spinal Cord Tumors. New York:
Thieme; 1996.
Klekamp J, Samii M. Extramedullary tumors. In: Klekamp J, Samii M,
eds. Surgtry ofSpinal Tumors. Berlin: Springer; 2007:143-320.
Klekamp J, Samii M. Intramedullary tumors. In: Klekamp J, Samii M,
eds. Surgtry ofSpinal Tumors. Berlin: Springer Verlag; 2007:120-131.
McCormick PC, Torres R, Post KD, et al. Intramedullary ependymoma
of the spinal rord.j Neurosurg. 1990;72(4):523-532.
Please go to ExpertConsult.rom ro view the romplete list of references.