CHAPTER

142 Alfred T. Ogden

Paul C. McCormick

Intradural, Extramedullary
Spinal Tumors

BACKGROUND direct involvement of the nerve root of origin or compression

of an adjacent nerve root. Lesions that cause symptoms by
OVERVIEW spinal cord compression, such as meningiomas,12,14,15,25 or
that indirectly involve multiple nerve roots, such as myxopap-
Since the development of routine successful surgery for intra- illary ependymomas,26 induce a pain syndrome that is more
dural, extramedullary spinal tumors following the pioneering central, dull, and nonspecific, akin to that generated by
work by Frazier,5 Elsberg,4 and Cushing,2 removal of these intramedullary lesions. In cases of spinal cord compression,
tumors has been considered to be among the most rewarding pain is likely to be accompanied by neurological symptoms. In
operations in neurosurgery. Although tumors in the intradural general, neurological symptoms will occur in a dermatomal
extramedullary location encompass a range of histologies, they pattern, a spinal pattern, or some combination, depending on
are almost always benign and well encapsulated and, as such, the pathoanatomical relationships of the tumor. The classic
are typically amenable to surgical cure. Usually, improvement syndrome of stepwise progression through segmental, hemi-
in neurological symptoms rapidly follows the decompression of cord, and transverse cord dysfunction is seldom seen today
neural elements from tumor removal. Given a common thera- because of earlier diagnoses enabled by physician awareness
peutic goal of gross-total macroscopic resection (GTMR) and the widespread availability of magnetic resonance imag-
regardless of histological type, the traditional categorization ing (MRI).
based on anatomy rather than histology is still an appropriate
paradigm for treatment of this group of tumors. Moreover, an
anatomic understanding of their relationship to meningeal lay- PREOPERATIVE EVALUATION AND IMAGING
ers and neural elements forms the basis of useful principles to
effect their safe removal. Complete imaging is an important prerequisite to developing a
coherent operative plan. A contrast-enhanced MRI is an essen-
tial starting point. The vast majority of intradural, extramedul-
EPIDEMIOLOGY
lary lesions, including nerve sheath tumors, meningiomas, and
Intradural extramedullary tumors by definition form outside ependymomas, have similar imaging characteristics on MRI,
of the spinal cord and inside its dural coverings and arise from appearing hypointense to isointense to neural tissue on
the range of tissues within that anatomic space: nerves and T1-weighted sequences and enhancing homogenously after
nerve roots (neurofibromas, schwannomas, and paragan- contrast administration. Despite these commonalities, distin-
gliomas), arachnoid and dura (arachnoid cysts, meningiomas), guishing features can sometimes be gleaned that may suggest
blood vessels (hemangioblastomas), and ectopic tissues (der- certain histopathologies (Fig. 142.1). A close association with
moids, epidermoids, lipomas, teratomas, ependymomas, the dura or the presence of a dural tail suggests a meningioma.
metastases). In adults, the vast majority of intradural extramed- A dumbbell-shaped lesion traversing a neural foramen suggests
ullary tumors are nerve sheath tumors, meningiomas, or nerve sheath origin. For nerve sheath tumors with extensive
ependymomas of the filum terminale. In children, menin- extraforaminal components, computed tomography (CT) and
giomas and schwannomas are extremely rare and are sup- plain X-rays may be required to evaluate the extent of bony
planted in their rank of incidence by ectopic lesions associated destruction and the potential for postresection instability.
with dysembryogenesis. When MRI is ambiguous in defining whether the intracanalicu-
lar portion of a dumbbell tumor is intradural, CT myelography
can be revealing. Lack of contrast enhancement is suggestive of
PRESENTATION
rarer lesions such as dermoid/epidermoids, cavernous malfor-
The most common presenting symptom of an intradural, mations, and arachnoid cysts. Fat-suppressed MR sequences
extramedullary tumor is pain. In the case of nerve sheath can help to distinguish between intralesional lipid content,
tumors,13,23,24 the pattern is often dermatomal, reflecting hemorrhage, and cyst fluid.

1535

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 1536 Section XIII Tumor and Osteomyelitis

Intradural Extramedullary Tumors: Algorithm of preoperative imaging

Contrast-enhancing lesion Non-enhancing lesion

Dural base/tail Dumbbell Unclear intradural +Fat-suppression -Fat-suppression

Component

Meningioma Clear intradural Consider Dermoid Cavernous malformation

component CT myelogram Epidermoid Arachnoid cyst
Lipoma
Schwannoma
Meningioma Nerve sheath tumor
Filum ependymoma
Hemangioblastoma
Metastasis

Large extraforaminal component

With extensive bony erosion

CT, upright X-rays to assess

Need for stabilization

Figure 142.1. Flow chart illustrating likely histological diagnoses of intradural extramedullary spinal
tumors based on imaging characteristics on contrast-enhanced magnetic resonance imagining. CT com-
puted tomography.

CLASSIFICATIONS, INDICATIONS, 1 and are more common in patients with plexiform neurofibro-
AND CONTRAINDICATIONS mas and a history of irradiation. Very rarely do they involve the
spine.7
NERVE SHEATH TUMORS The treatment of choice for nerve sheath tumors is surgical
excision. Surgery is indicated for any symptomatic lesion and/
Nerve sheath tumors arise from supporting cells adjacent to or lesions exhibiting spinal cord compression on MRI. Since
neurons within spinal roots and nerves. Schwannomas account these are slow-growing tumors, small, incidentally discovered
for around 85% of these tumors and are the most common of lesions can be followed with serial imaging studies until growth
all intradural, extramedullary spinal tumors.24 They arise from is documented or until symptoms occur. Although a likely strat-
myelin-producing Schwann cells and grow tangentially to nerve egy for patients with NF and multiple lesions, observation of
fibers in two characteristic collagen fiber bundling patterns: sporadic lesions carries the risk attendant to any presumed
Antoni A and Antoni B. Neurofibromas, which account for diagnosis. In particular, the inability to distinguish conclusively
around 15% of nerve sheath tumors,23 lack this histological on MRI between nerve sheath tumors and filum ependymomas,
organization, consisting of fibrous cell bundles admixed with which are likely better served by early surgical intervention,
nerve fibers. There is still debate over their cell of origin. makes the removal of all lesions associated with lumbar nerve
The typical nerve sheath tumor occurs sporadically and roots imperative. The decision to operate must, of course, be
presents in the fourth decade of life without apparent predilec- weighed against the general health and age of the patient, as
tion for gender or spinal level.23,24 Because of associations with these may be relative contraindications to surgery.
neurofibromatosis (NF), however, any patient presenting with a In rare cases of MNSTs affecting the spine, surgery is still
nerve sheath tumor should be examined for the stigmata of NF indicated but must be performed with the goal of achieving
and counseled regarding this potential diagnosis. Neurofibromas wide surgical margins and a follow-up plan for adjuvant ther-
are associated with neurofibromatosis type 1 (NF 1), whereas apy. Malignant degeneration of nerve sheath tumors is not
schwannomas are associated with neurofibromatosis type 2 uncommon in the NF population and must be considered in
(NF 2). Multiple neurofibromas or schwannomas are pathog- any lesion that demonstrates rapid growth on sequential
nomonic for their respective NF subtypes. imaging.
Approximately 3% of peripheral nerve sheath tumors
exhibit gross or microscopic evidence of invasiveness and a
MENINGIOMAS
high mitotic index. Such tumors are lumped into the classifica-
tion of malignant nerve sheath tumor (MNST) regardless of Meningiomas are the second most common intradural,
their other histological features. MNSTs are associated with NF extramedullary spinal tumor. The incidence of meningiomas

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 Chapter 142 Intradural, Extramedullary Spinal Tumors 1537

peaks between the ages of 50 and 65, they are seen four to nine benign lesions that have presumably existed since birth, the
times more frequently in women as in men,14,15 and they are role for surgical resection, particularly in adults, should be
most common in thoracic spine.25 Meningiomas arise from heavily influenced by the presence and progression of neuro-
cells within the arachnoid villi and usually have dural attach- logical symptoms. In adults, incidentally discovered lesions may
ments. The increased density of arachnoid villi near dural root not require treatment.
sleeves may partially explain the lateral position of most men- In the face of progressive symptoms, surgery is indicated.
ingiomas, although dorsal and ventral lesions occur as well. Operative planning must take into account any dysraphism
Several histological subtypes are seen in the spinal cord, psam- present as well as mass resection. Although benign, these
momatous being the most common. With the rare exceptions tumors are often densely adherent to neural structures and
of angioblastic and atypical subtypes (which tend to occur in complete surgical excision without neurological injury is seem-
younger patients), histological variation has no bearing on ingly impossible. In such cases, complete tumor removal is nei-
treatment or prognosis. Surgical excision is the treatment of ther safe nor necessary for excellent long-term outcomes.17
choice, although, like nerve sheath tumors, meningiomas are
generally slow-growing lesions, making the decision to operate
OTHERS
a balance between the extent of neurological symptoms, the
degree of radiographic neural compression, and the age and Hemangioblastomas can be entirely extramedullary; indeed,
overall health of the patient. extramedullary hemangioblastomas comprise up to 30% all
hemangioblastomas found in patients with von HippelLindau
disease.16 Lacking the appropriate milieu to generate charac-
EPENDYMOMAS
teristic intramedullary cysts, these tumors can masquerade as a
Ependymomas are seen in association with the lumbosacral lumbar nerve root schwannoma on MRI. Other histological
nerve roots either as intramedullary ependymomas of the conus subtypes in the intradural extramedullary compartment are
or extramedullary ependymomas of the filum. Only the latter rare. Paragangliomas and systemic metastases, particularly mel-
are considered here. Filum ependymomas are thought to arise anoma, can occur in association with nerve roots and can also
from ectopic rests of ependyma and can be of any histological be indistinguishable from more common pathology on preop-
subtype. Still, the overwhelming majority of filum ependymo- erative imaging.
mas are of the myxopapillary subtype, which rarely occurs
outside of this location. Although frequently adherent to lum-
bosacral nerve roots, myxopapillary ependymomas are usually OPERATIVE TECHNIQUE
separated from the conus by a margin of normal filum. As such,
they are true extramedullary lesions. APPROACHES
Although schwannomas and meningiomas are very rare in
children and young adults, around 20% of myxopapillary
Overview
ependymomas occur in the first two decades of life; the overall In our experience, the majority of intradural, extramedullary
mean age of presentation is in the mid-thirties.26 Because of tumors can be removed through a standard dorsal midline
their potential to metastasize along cerebrospinal fluid (CSF) approach. The midline posterior approach provides the most
spaces, their lack of true encapsulation, and their unpredict- familiar approach to normal neural anatomy prior to tumor
able growth rate, filum ependymomas should be removed in a resection and affords the earliest opportunity to decompress
timely manner. With gross-total resection, cure rates for all spi- the spinal cord. This approach is advised for all tumors in the
nal ependymomas range from 91% to 100%.21 Myxopapillary lumbar region and thoracic or cervical tumors lying dorsal or
ependymomas are associated with higher rates of both subtotal dorsolateral to the spinal cord. Ventrolateral and ventral tumors
resection and recurrence compared to other ependymoma can often be accessed dorsally as well, provided that a sufficient
subtypes, however, because of their immediate access to the surgical corridor exists. Often this corridor has been made by
lumbar cistern and their tendency to adhere to lumbosacral the tumor itself, as the slow growth of an eccentrically located
nerve roots.1,26,27 tumor has gradually pushed the spinal cord to one side. Small
thoracic lesions without sufficient lateral displacement of the
CONGENITAL TUMORS spinal cord are sometimes better resected via a lateral approach
such as the retropleural approach. Purely ventral lesions of the
Dermoids, epidermoids, lipomas, and teratomas are thought to cervical and high thoracic cord may require anterior approaches.
be congenital lesions resulting from errors in the cleavage Supra-axial lesions that cannot be managed from a dorsal
between neural ectoderm and cutaneous ectoderm that occurs approach may require far lateral or transoral exposures.
between the third and fifth week postconception. Rarely, iatro-
genic transplantation of epidermal cells after lumbar puncture
Dorsal Midline Approach
or myelomeningocele repair lead to tumor formation as well.
These tumors are typically discovered in childhood, occur pre- This approach was described originally by Gowers and Horsley
dominately in the lumbosacral region, and may be seen in asso- who, in 1887, described the first successful removal of an intra-
ciation with spinal dysraphisms such as dermal sinus tracts, dural extramedullary tumor.6 Refinements of this technique
tethered cords, and split cord malformations. The incidence of include the use of the operating microscope and microsurgical
dermoids and epidermoids decreases dramatically with increas- techniques, advances in anesthesia, and the advent of neuro-
ing age, and they are very rarely diagnosed in adulthood. Such physiological monitoring. We use a standard operating table
lesions account for 5% to 17% of all spinal tumors in surgical with the patient prone on a Wilson frame or cushioned bol-
series of pediatric patients with spinal tumors.17 As slow-growing, sters. A Mayfield head holder is used for lesions above T5. After

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 1538 Section XIII Tumor and Osteomyelitis

standard intubation, an arterial line and Foley catheter are Purely ventral meningiomas and nerve sheath tumors of the ros-
placed, and a monitoring array with MEP, SSEP, nerve stimula- tral spine, between C3 and T2, can be resected using a standard
tion, and free running EMG capabilities is applied. anterior cervical approach and corpectomy.20 Ventral supra-
Preoperative antibiotics and steroids are given. After stan- axial tumors may require lateral skull base approaches. Purely
dard prepping and draping, a midline incision is made and a ventral meningiomas from T2 to T4 may require open thoracic
subperiosteal dissection of the soft tissues off of the spinous exposures such as the trapdoor approach.19
processes and lamina is performed. Localization is confirmed
with a portable X-ray or fluoroscopy. A laminectomy is per-
INTRADURAL COMPONENT
formed with its width and laterality tailored to tumor anatomy.
Sometimes a unilateral partial facetectomy is necessary to A straight midline durotomy is usually adequate and is conve-
obtain adequate exposure, particularly for access to lateral or nient to close; paramedian or curvilinear incisions can be help-
ventrolateral lesions. Dural exposure proceeds with meticulous ful in come cases, however. The arachnoid is preserved during
cauterization of epidural vessels and bone waxing. Complete durotomy to prevent bleeding from epidural veins induced by
hemostasis is required prior to durotomy to maintain a clean a loss of adjacent CSF pressure and to preserve the tumors
intradural operative field. Ultrasonic confirmation of tumor relationship to this meningeal layer. The dura is tented over a
location is useful at this point to define the limits of the dural quarter-inch cottonoid lining the epidural space at the limits of
opening. We have observed lumbar lesions to shift position the laminectomy and secured to the paravertebral muscles with
relative to preoperative imaging. 4.0 silk sutures.
At this point, the tumors relationship to the arachnoid is
explored. Intradural nerve sheath tumors and ependymomas
Other Approaches
will lie within the arachnoid (Fig. 142.2A and C) and can often
Other approaches may be either desired or necessary as a pri- be distinguished prior to resection based on association with
mary or adjunct exposure. The lateral extracavitary approach is the filum or an individual nerve root. Nerve root stimulation is
useful for dumbbell tumors from T4 to L5 with extensive extra- often a useful adjunct to confirm visual assessments and to
foraminal extension.18 We prefer this to retroperitoneal and assess the functionality of nerve roots. Meningiomas will usually
retropleural approaches because it reduces the likelihood of be separated from the neural elements by a clear arachnoid
CSF fistulae to the peritoneal or pleural spaces. For cervical and plane (Fig. 142.2B and D).
high thoracic lesions with large extraspinal components, we opt The arachnoid overlying the exposed tumor capsule is dis-
for a staged operation, where the intradural portion of the sected free, and the exposed tumor capsule is cauterized. This
tumor is resected first along with accessible portions of the begins to interrupt the tumors blood supply and shrink down
extraforaminal component. With the spinal cord decompressed, the tumor mass. While small tumors may be removed en bloc,
there is ample time to allow the dural and fascial planes to heal larger tumors require internal debulking prior to dissection of
prior to the excision of the extraforaminal residua, which can the capsule from neural elements. Debulking is usually best
be pursued through an anterior or a transaxillary approach. achieved with an ultrasonic aspirator. From the dorsal approach,

A C

Figure 142.2. Sagittal contrast-

enhanced MRIs of a filum
ependymoma (A) and a lumbar menin-
gioma (B) and the corresponding
intraoperative images of each. The
ependymoma, (e) is found inside the
arachnoid originating from the filum
terminale (yellow arrows) (C). The men-
ingioma (m) is attached to the dura
(d) and is separated from the nerve
B D
roots (nr) by an arachnoid layer (D).

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 Chapter 142 Intradural, Extramedullary Spinal Tumors 1539

TABLE 142.1

Relationship to Relationship to Spinal Nonfunctional Neural

Type Dura Canal Origin Element Surgical Plan
I Intradural Intraspinal Dorsal or ventral Dorsal or ventral nerve Sacrifice nerve root of
nerve root root of origin origin, spare paired
nerve root
II Intradural with Extraspinal extension Dorsal or ventral Entire nerve root Sacrifice entire nerve
extradural via nerve root sleeve nerve root (anterograde spread root/spinal nerve
component to spinal nerve)
III Extradural Paraspinal extension Proximal spinal Fascicle of origin Isolate tumor from
(foraminal) nerve fascicle spinal nerve
IVa Extradural Paraspinal with Proximal spinal Fascicle of origin Isolate tumor from
intraspinal extension nerve fascicle spinal nerve
IVb (NF-2) Intradural Paraspinal with Proximal spinal Entire nerve root Sacrifice entire spinal
extension intraspinal extension nerve fascicle (retrograde spread to nerve and nerve
dorsal and ventral roots
spinal nerve roots)

unilateral sectioning of the dentate ligament and gentle rota- all patients in these series with postoperative deficits had cervi-
tion of the spinal cord to achieve adequate exposure may facili- cal schwannomas.11,13,23,24
tate excision of lateral lesions. Dissection of the tumor from A theoretical framework for determining whether the surgi-
neural elements is usually straightforward, alternating with seg- cal goals should include preservation of the nerve or nerve root
mental cauterization of the tumor capsule and division of feed- of origin lies in ascertaining the segment of the nervenerve
ing vessels. Rarely is the blood supply of the tumor and spinal root complex from which the tumor arises (Table 142.1). The
cord shared. Removal of each of the main histological types is anatomical transition from spinal nerve root to spinal nerve is
nuanced at this point and bears special consideration. characterized by a separate entry of dorsal and ventral roots
Filum ependymomas should ideally be resected en bloc, as into the dural root sleeve (Fig. 142.3A) and the merging of
there is some evidence that piecemeal resection of ependymo- these nerve roots just distal to the dorsal root ganglion (Fig.
mas increases recurrence rates.26,27 The tumor is carefully freed 142.3B). At the ultrastructural level, a thinning of the dural
from adjacent nerve roots, and the filum is identified visually sleeve into epineurium and the separation of nerve fibers into
and tested with a neurostimulator. The filum is cauterized fascicles by perineurium characterize this transition. Tumors
above and below the tumor and divided, and the tumor is care- can arise anywhere along this segment, and surgical resection is
fully rotated out of the canal. En bloc resection is often not nuanced according to this site of origin and subsequent growth
possible to achieve safely in larger tumors for various reasons. pattern.
The tumor may lack sufficient internal integrity and fall apart Type I tumors are termed intradural, intraspinal (Fig. 142.4).
with even gentle manipulation. The tumor may be simply too They arise from a dorsal or ventral nerve root lacking epineu-
large to tease out without putting unacceptable amounts of rium or perineurium. In such cases, the nerve root of origin
traction on overlying nerve roots. The tumor may be hopelessly (usually dorsal) is inseparable from tumor, nonfunctional, and
stuck to nerve roots; in such cases, subtotal resection is prefer- unsalvageable. It may, however, be distinguishable from its
able to over-manipulation. paired nerve root (usually ventral) within a common arachnoid
Successful meningioma surgery is facilitated by maintenance sheath. The paired nerve root may be functional and should, if
of an arachnoid plane. If preserved, it will effectively guide the possible, be identified and spared. This may not be feasible
surgeon between tumor and spinal cord and aid in the dissec- with larger tumors. Type II tumors are intradural with extraspi-
tion of nerve roots off of the tumor capsule. When the tumor nal extension through the root sleeve (Fig. 142.5). These
resection is near completion, a decision must be made regard- tumors invade the extraspinal peripheral nerve, entailing inti-
ing the tumors dural attachment. Resection of associated dura macy with fibers originating from both the nerve root of origin
followed by patch-grafting of the dural defect is perhaps desir- and its paired nerve root. Thus root preservation is not neces-
able, but may be difficult or impossible to achieve in a water- sary or possible, regardless of tumor size. Type III tumors arise
tight fashion for tumors with lateral or anterior attachments. from an extradural proximal spinal nerve and grow away from
The availability of numerous dural substitutes and caulking the spinal canal into the paraspinal space (Fig. 142.6). As such,
agents gives the surgeon many unproven options to aid closure. they are really peripheral nerve tumors for which the fascicle of
Whether dural resection reduces recurrence rates over dural origin can be isolated from the rest of the spinal nerve. Type IV
cauterization or split thickness resection is debatable and must tumors arise from the proximal spinal nerve as well but grow
be weighed against the perceived likelihood and morbidity of a into the spinal canal where they remain extradural (IVa)
CSF leak. (Fig. 142.7) or invade through the root sleeve to become intra-
Nerve sheath tumors often require sacrifice of the nerve dural (IVb). In the first case, resection should follow the princi-
root of origin in order to be removed. Often this requires the ples of peripheral nerve tumor surgery in which the tumor can
sacrifice of a spinal nerve or nerve root. Surgical series have and should be isolated from undiseased fascicles. In the second
reported permanent loss of function after nerve root section- case, which has only been seen in patients with NF-2, the tumor
ing in 2% to 4% of patients with nerve sheath tumors. Notably, has invaded the intradural space growing into both dorsal and

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 1540 Section XIII Tumor and Osteomyelitis

Figure 142.3. Cadaver dissection of

a cervical nerve root. Separate entry of
dorsal (dr) and ventral (vr) nerve roots
into the dural sleeve are seen (A). Dor-
sal and ventral nerve roots merge after
A B
the dorsal root ganglion (drg) (B).

ventral nerve roots. In such cases, neither nerve root can be The muscle is loosely approximated with a running 0 absorbable
spared. monofilament synthetic suture (Biosyn) and the dorsal fascia
closed with interrupted 0 absorbable braided synthetic suture
(Vicryl) in a watertight fashion. The skin is closed with a subcu-
CLOSURE AND RECONSTRUCTION
taneous 2-0 absorbable braided synthetic suture followed by a
After the tumor is removed and hemostasis is achieved, the suba- running 3-0 monofilament nylon suture for skin closure.
rachnoid space is copiously irrigated with warm saline and the Occasionally the combination of bone exposure and bone
dura is closed primarily with a running locked 4.0 silk suture. erosion caused by nerve sheath tumors causes instability that

A B C

D E

Figure 142.4. Type I Schwannoma. Contrast-enhanced T1-weighted MRI shows a lesion consistent with
an entirely intradural schwannoma at the level of C5 (A and B). Type I tumors arise from a single root (usu-
ally dorsal) and the paired root is associated but not invaded by tumor (C). Intraoperative images (D and E)
show that it is sometimes possible to save the paired nerve root (pnr).

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 Chapter 142 Intradural, Extramedullary Spinal Tumors 1541

A B

Figure 142.5. Type II Schwannoma. Type II tumors arise for a single nerve root but grow through the
neural foramen and invade the peripheral spinal nerve (A). After extension through the neural foramen
(nf), the peripheral portion of this tumor (t) is intimate with fibers from both dorsal and ventral nerve roots;
therefore, both nerve roots can be sacrificed (black arrow) (B).

should be addressed in the same operation as tumor removal. POSTOPERATIVE MANAGEMENT

Cases in which the entire lateral mass at a cervical level or the
entire facet complex at the thoracolumbar junction has been We typically require patients to remain level in bed for 36 to 48
destroyed are obvious examples that demand immediate stabi- hours after surgery with routine spinal drainage reserved for
lization after tumor resection. Most cases are less clear-cut and patients with anterior approaches only. All patients are ordered
require an individualized assessment that takes into account for lower extremity sequential compression devices, incentive
the patients age, the innate flexibility of the patients spine, spirometry, Foley catheters, and patient-controlled analgesia
and the patients activity level. Relative instability can often be (PCA). During this time, patients are permitted to lie on their side
addressed at a later date without any serious consequences. or stomach and may elevate their upper bodies 10 to 15 degrees

A B

C D

Figure 142.6. Type III Schwannoma. Type III tumors arise from a single fascicle within the extradural
proximal spinal nerve and grow radially into the paraspinal space (A and B). The anatomic separation of fas-
cicles by perineurium permits tumor dissection that preserves other fascicles within the nerve of origin. The
tumor (t) is shelled out of peripheral nerve (n), which is distended but functional (C and D).

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 1542 Section XIII Tumor and Osteomyelitis

A B

Figure 142.7. Type IVa Schwan-

noma. Type IVa tumors arise from the
extradural proximal peripheral nerve
as well, but growth into the spinal
canal, causing neural compression (A
and B). Type IVa tumors (t) remain
extradural (d) and are thus separated
from the nerve roots and spinal cord
C D
(C and D).

for meals. Patients are mobilized on postoperative day 2 and OUTCOMES AND FOLLOW-UP
are discharged between postoperative days 3 and 5.
Overall, the outcomes from intradural extramedullary tumors
are excellent. Most patients experience an improvement in
COMPLICATIONS functional status and long-term remission after gross macro-
scopic resection without any adjuvant therapy. Neurological
Serious peri-operative complications such as a major postop- deficits often resolve partially or completely in the immediate
erative neurological deficit, postoperative hematoma, or postoperative period, with further progress made with physical
thromboembolus from activity restriction are exceedingly rare. and occupational therapy in the months following surgery.
A mild exacerbation of preexisting neurological deficits is For nerve sheath tumors, pain is the symptom that most con-
unusual and typically resolves in the immediate postoperative sistently responds to surgery followed by weakness and then by
period. Dysesthesias after nerve root sacrifice can be nettle- sensory loss.11,13,23,24 Although gross-total resection results in a
some and may theoretically be avoided in extraforaminal cure for most patients, precise recurrence rates after surgery
tumor surgery by root sectioning proximal to the dorsal root are difficult to assess because of a lack of studies with long-term
ganglion. A variety of medications including anticonvulsants follow-up. One population study from Finland found a 10.7%
and tricyclics may be effective in dealing with this neuropathic 5-year recurrence rate in sporadic cases of spinal schwannoma
pain syndrome. A CSF leak or pseudomeningocele can occur and a 39.2% 5-year recurrence rate in patients with NF-2.13
in the immediate postoperative period or in a delayed fashion. These relatively high numbers, in conjunction with the authors
A CSF leak can often be remedied by wound oversewing with observation of recurrences in cases of complete tumor resec-
or without spinal drainage. Pseudomeningoceles sometimes tion and transection of the offending nerve root, dictate that
respond to CT-guided percutaneous drainage with or without all patients should be followed with postoperative serial MRIs
spinal drainage. Both CSF leak and pseudomeningocele may for several years.
require reoperation, however, as can infections, which occur at Outcome after meningioma surgery is linked to the patients
a rate similar to that of other noninstrumented spine cases. preoperative neurological condition. Although surgery can
Laminectomy carries a delayed risk of spinal instability in the exacerbate presenting symptoms, such setbacks are usually
cervical, lumbar, and thoracolumbar spine that is related to temporary and respond to therapy. Surgical series report sig-
the number of levels exposed and to the extent of facetec- nificant and often dramatic recovery of neurological function
tomy.10,22 Although the degree of bony sacrifice should be in most patients. For example, 80% to 90% of nonambulatory
minimized, especially outside of the middle and upper tho- patients are reported to regain the ability to walk after menin-
racic spine, it should never compromise the exposure needed gioma removal12,14,15,25 and one series reported complete neu-
to remove the tumor safely. rological recovery in 46% of paraplegic patients.8

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 Chapter 142 Intradural, Extramedullary Spinal Tumors 1543

The likelihood of recurrence of spinal meningiomas is must be followed with serial imaging for several years to identify
closely tied to the macroscopic extent of resection, with GTMR recurrences regardless of the surgeons confidence in the
the goal of surgery. Surgical series report recurrence rates of extent of resection.
totally resected meningiomas at 1.3%,12 6%,25 and 29.5%13 with
average follow-up periods ranging from 5 to 15 years. Rates of
GTMR are reported from 89% to 98% with subtotal resection
associated with recurrent tumors, arachnoid scarring, en plaque REFERENCES
morphology, malignant histological features, and anterior loca- 1. Chang UK, Choe WJ, Chung SK, Chung CK, Kim HJ. Surgical outcome and prognostic
tion.12,14,15,25 As with nerve sheath tumors, postoperative serial factors of spinal intramedullary ependymomas in adults. J Neurooncol 2002;57:
133139.
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