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Spinal cord tumors

Authors Section Editors Deputy Editor

William C Welch, MD, FACS, FICS Jay S Loeffler, MD April F Eichler, MD, MPH
David Schiff, MD Patrick Y Wen, MD
Peter C Gerszten, MD, MPH, FACS

Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2013. | This topic last updated: ago 28, 2012.

INTRODUCTION — Spinal cord tumors can occur within or adjacent to the spinal cord. They are considered to be
intraaxial in location and can be either primary or metastatic. Primary spinal cord tumors account for 2 to 4 percent
of all primary central nervous system (CNS) tumors, one-third of which are located in the intramedullary
compartment.

Spinal cord tumors can be classified according to their anatomic location (figure 1):

Intramedullary — Intramedullary tumors arise within the spinal cord itself. Most primary intramedullary
tumors are either ependymomas or astrocytomas. Metastases are being recognized with increasing
frequency, primarily because of improvements in imaging modalities.

Intradural-extramedullary — Tumors arising within the dura but outside the actual spinal cord are termed
intradural-extramedullary. The most common tumors in this group are meningiomas and nerve sheath
tumors. (See "Meningioma: Clinical presentation and diagnosis" and "Intradural nerve sheath tumors".)

Extradural — Extradural tumors are usually metastatic and most often arise in the vertebral bodies.
Metastatic lesions can cause spinal cord compression either by epidural growth that results in extrinsic
spinal cord or cauda equina compression or less frequently by intradural invasion. (See "Clinical features and
diagnosis of neoplastic epidural spinal cord compression, including cauda equina syndrome".)

The relative frequency of primary spinal tumors is illustrated by a Surveillance, Epidemiology, and End Results
(SEER) and National Program of Cancer Registries analysis that included 11,712 cases identified over a four year
period [1]. Of these, 9136 were benign (78 percent) and 2576 were malignant (22 percent). By histology, the three
most common tumors were meningioma, tumors of spinal nerves, and ependymoma (33, 27, and 21 percent,
respectively).

The clinical manifestations and general approach to spinal cord tumors will be reviewed here.

PRESENTATION

Symptoms — Tumors within or extrinsic to the spinal cord can cause symptoms through disruption of normal
neural elements and pathways, producing both local and distal effects.

The most frequent local effect is pain that causes nocturnal awakening. Patients often describe this pain as a
gnawing and unremitting [2]. The site of this may provide an indication of the anatomic location of the tumor.

Neurologic dysfunction distal to the lesion is due to interruption of ascending and descending spinal cord pathways.
The most common sequelae are sensory dysesthesias and muscular weakness, especially of the iliopsoas
musculature. Patients often report progressive difficulty in ambulation. Severe distal sensory loss and sphincter
dysfunction also may occur. Although neurologic manifestations may begin unilaterally, they can progress to
involve both sides of the spinal cord and thereby produce bilateral symptoms and signs.

History — A prior history of cancer may suggest a diagnosis of metastasis to the spinal column, which may cause
axial or radiating pain. Referred pain, eg, to the shoulder or neck, is also common with spinal metastases [3].

The spine is a common metastatic site for many tumor types. As an example, vertebral metastases have been
found at autopsy in 90 percent of patients who died of prostate cancer, 74 percent with breast cancer, and 45
percent with lung cancer [4,5].

Physical examination — A thorough physical examination is necessary to define probable sites of tumor
involvement, document preoperative neurologic deficits, and determine progressive neurologic deterioration. An
assessment of the patient's ambulatory status is also necessary since this carries important prognostic
significance.

Imaging — Magnetic resonance imaging (MRI) of the spine is currently the diagnostic study of choice, providing
excellent delineation of the spinal cord and surrounding structures. Almost all intrinsic spinal cord tumors and
metastases enhance with gadolinium [6]. (See "Clinical features and diagnosis of neoplastic epidural spinal cord
compression, including cauda equina syndrome", section on 'Radiologic confirmation'.)

INTRAMEDULLARY TUMORS — The majority of intramedullary primary spinal cord tumors are gliomas. The term
glioma refers to a tumor bearing a histologic resemblance link to normal glial cells. The major types of glial tumors
are ependymomas, astrocytomas, and oligodendrogliomas, and mixtures of these cell types are occasionally seen
within a single tumor. Spinal cord gliomas are rare compared to cerebral lesions, probably because of the relative
paucity of glial tissue in the spinal cord.

The distribution of histologic subtypes of spinal gliomas also differs considerably from intracranial gliomas. Although
glioblastomas account for the majority of intracranial gliomas, they comprise only 5 percent of spinal lesions. In
contrast, ependymomas comprise approximately 60 to 80 percent of spinal gliomas compared to 3 percent of
intracranial gliomas [7]. (See "Classification of gliomas" and "Diagnosis and classification of low-grade gliomas".)

Ependymomas — Ependymomas are intramedullary tumors that may be located anywhere along the spinal cord.
Approximately one-half occur in the lumbosacral spinal cord or filum terminale; the other 50 percent occur
anywhere in the cervical or thoracic spinal cord (image 1) [8]. Extraneural metastases are uncommon [9]. (See
"Ependymoma".)

In the World Health Organization (WHO) classification of brain tumors, ependymal tumors are divided into four
major groups: ependymoma, myxopapillary ependymoma, subependymoma, and anaplastic ependymoma [10].
Ependymomas are subdivided into cellular, papillary, clear cell, and tanycytic types.

Ependymoma — Ependymomas are the most common intramedullary tumors in adults, with a peak age at
presentation between 30 and 40 years [10]. The cellular subtype is most common.

Patients often have localized pain for months to years prior to developing other symptoms. Neurologic deficits may
include lower extremity spasticity, loss of pain and temperature sensation, lower extremity and truncal sensory
diminution to light touch and vibration, and gait ataxia. Ependymomas tend occur centrally within the cord,
expanding it symmetrically as they grow [11]. The spinal cord may be expanded along several segments, and a
tumor-associated cyst (syrinx) is common [12]. These lesions generally enhance intensely on MRI.

Optimal management consists of gross total resection by an experienced surgeon. Although these are infiltrative
tumors, a total or near-total resection can frequently be achieved without causing further neurologic deficits. There
are no randomized trials that have evaluated the role of adjuvant RT.

The most extensive data on the natural history and response to treatment come from an observational series of 126
patients with spinal ependymoma, treated over a 47-year period at six institutions [13]. The most frequent subtype
was cellular ependymoma, which accounted for approximately 60 percent of classified cases; approximately 70
percent were moderate grade. Myxopapillary ependymomas comprised 17 cases in this series (13 percent).

Treatment included complete resection in 63 cases (50 percent); in 12 of these (19 percent), postoperative RT was
given. Among patients in whom a partial resection or biopsy only was performed, 47 of 58 (81 percent) had RT as
part of their initial management. Median follow-up was approximately four years. Progression-free and overall
survival rates at 15 years were 35 and 75 percent, respectively. Multivariate analyses supported the importance of
complete resection, but did not demonstrate a benefit from adjuvant RT.

There is no proven role for chemotherapy in recurrent or metastatic spinal cord ependymomas. However, the
authors have occasionally seen disease stabilization in patients with advanced disease treated with carboplatin-
based regimens.

Myxopapillary ependymoma — Myxopapillary ependymomas are biologically and morphologically distinct

from other ependymomas. These tumors most commonly arise in the lumbosacral spinal cord and filum terminale.
Immunohistochemistry may be needed to differentiate myxopapillary ependymomas from either chordomas or
chondrosarcomas. (See "Chordoma and chondrosarcoma of the skull base".)

Myxopapillary ependymomas are slow-growing glial tumors that typically are found in young adults and are more
common in males. Myxopapillary ependymomas generally present with low back pain, with or without radicular
features [14].

Initial management of these tumors consists of laminectomy with attempted surgical resection. These tumors
frequently can be totally resected, and many patients are cured following gross total resection. However, some
patients recur locally or with leptomeningeal tumor dissemination as much as 20 years after the initial surgery.
Subtotal resection may be necessary, particularly in patients with unencapsulated tumors. In such cases, survival
may still be prolonged [15-17].

There are no randomized trials addressing the role of postoperative radiation therapy (RT), and observational studies
have given conflicting results [15-17].

The biology, natural history, and prognosis for myxopapillary ependymoma are illustrated by a series of 85 patients
gathered by the Rare Cancer Network [15]. The average age for the entire cohort was 37 years, and tumors were
more common in males than females (59 and 41 percent, respectively). The primary lesion was in the lumbosacral
spine or filum terminale in 45 cases (53 percent), while most of the remainder were in the thoracolumbar region.

All patients were initially managed with surgery, with gross total resection, partial resection, and biopsy only in 47,
51, and 2 percent of cases, respectively. Postoperative RT was given to 55 percent. The five-year progression-free
survival rates with surgery alone and surgery plus RT were 50 and 75 percent, respectively. Treatment failure was
seen in 24 patients and was predominantly local. Multivariate analysis showed a statistically significant decrease in
the incidence of recurrence in those receiving high-dose postoperative RT (≥50 Gy).

Anaplastic ependymoma — These high-grade ependymomas have anaplastic features on histological

examination (necrosis, mitosis, vascular proliferation, cellular pleomorphism, and overlapping of nuclei) [18]. These
tumors are relatively uncommon and comprised only 5 percent of spinal ependymomas in one large series [13].
Compared to lower grade ependymomas, anaplastic tumors appear to have a higher recurrence rate and poorer
survival.

Astrocytomas — Astrocytomas occur throughout the spinal cord. The pathologic features of spinal astrocytic
lesions are predictive of the biologic behavior and clinical course. Approximately one-half of spinal cord
astrocytomas are pilocytic and one-half are infiltrative astrocytomas [19]. (See "Diagnosis and classification of low-
grade gliomas".)

Pilocytic astrocytomas are well circumscribed and low grade with nonaggressive clinical behavior. On MRI
these tumors enhance intensely with gadolinium (image 2) [19-22].
 Diffuse fibrillary astrocytomas of the spinal cord usually appear as nonencapsulated lesions that enhance
minimally or heterogeneously on MRI. About one-third are histologically high-grade (anaplastic astrocytoma
or glioblastoma [GBM]).

There are no randomized trials defining the optimal approach to the management of these tumors. The most
extensive experience comes from a retrospective series of 136 patients (69 with pilocytic lesions and 67 with
infiltrative astrocytomas) treated at the Mayo Clinic over a 43 year period [19]:

At diagnosis, the median age was 35 years, and symptoms were present for a median of eight months. The
series included.

Gross total resection was feasible in 16 percent of patients, subtotal resection in 25 percent, and biopsy
only in 59 percent. There were no differences between pilocytic and infiltrative lesions in the extent of
resection.

Survival was significantly longer for patients with pilocytic tumors compared to those with infiltrative
astrocytomas (10-year survival 78 versus 17 percent). Even for patients with infiltrative lesions, there
appeared to be a plateau in the survival curve with some long-term survivors.

The most important factor associated with a better prognosis was low tumor grade (WHO grade I). Other
factors associated with a better prognosis included tumor location other than the cervical region, limited
extent of tumor involvement along the spinal cord, and longer (>180 days) duration of symptoms. Multivariate
analysis could not demonstrate an advantage for more aggressive surgical resection.

Postoperative RT did not affect outcomes in patients with pilocytic astrocytomas, although it did appear to
improve the outcome for infiltrative astrocytomas.

The role of chemotherapy in this disease is undefined. There are no reported trials of chemotherapy for spinal cord
astrocytomas, and in our experience, chemotherapy has been ineffective. However, there are small observational
series in the pediatric literature indicating that chemotherapy has at least some activity [23-26].

Management of ependymomas and astrocytomas — The initial step in the management of a patient with a
presumed primary intramedullary spinal cord tumor is resection to the maximum extent possible. A total or near-
total resection can frequently be achieved without causing further neurologic deficits.

Given the rarity of spinal cord diffuse fibrillary astrocytomas, there are no randomized trials to guide
recommendations for subsequent treatment. Our approach is to use fractionated RT for low-grade tumors that are
incompletely resected and for all high-grade tumors. There is no established role of chemotherapy in the initial
management of these patients.

Metastases — Intramedullary spinal cord metastases are rare, although the increased use of MRI has resulted in
more frequent recognition of such lesions (image 3) [27-29]. About one-half of cases are associated with lung
cancer. Breast cancer, renal cell carcinoma, Iymphoma, and melanoma are other tumor sites that metastasize to
the spinal cord.

Intramedullary metastases are usually observed in patients with widespread metastatic disease. The majority of
patients also have brain and lung metastases, while leptomeningeal metastases are seen in approximately 25
percent [27].

Patients typically present with weakness, numbness, and pain. The Brown-Sequard syndrome of hemicord
dysfunction is a common initial finding and may raise suspicion of this diagnosis as compared to the more common
epidural spinal cord compression. MRI is generally diagnostic. A CT-myelogram can be useful in patients who
cannot undergo MRI. (See "Anatomy and localization of spinal cord disorders", section on 'Brown-Sequard (hemi-
cord) syndrome'.)
Management of intramedullary metastases generally consists of fractionated RT, which usually maintains but does
not improve the pretreatment level of neurologic function. As with the treatment of brain metastases and epidural
spinal cord compression, corticosteroids are used to diminish the effects of radiation-induced edema until RT is
completed [27]. (See "Treatment and prognosis of neoplastic epidural spinal cord compression, including cauda
equina syndrome".)

INTRADURAL EXTRAMEDULLARY TUMORS — Both meningiomas and nerve sheath tumors (schwannomas and
neurofibromas) can develop in the intradural extramedullary spinal compartment (figure 1).

Meningioma — Meningiomas can arise from arachnoidal cells anywhere along the neuraxis. They are
occasionally found in association with neurofibromatosis [30-32]. Approximately 90 percent of the tumors occur
within the cranial fossa. (See "Meningioma: Clinical presentation and diagnosis".)

Spinal meningiomas most commonly occur within the thoracic spine (image 4). The tumors are frequently adherent
to the spinal dura, requiring dural resection for complete removal, and also grow along intradural and extradural
components of the nerve roots.

Spinal meningiomas are typically slowly growing, invasive lesions and may remodel or erode bone. Calcifications
may be suggestive of the histologic diagnosis of meningioma [33].

Pathologically, spinal meningiomas demonstrate the same features seen with intracranial lesions. Meningiomas
can have areas of increased cellularity or vascularity, xanthomatous components, and areas of hemorrhage. Rarely,
the tumors may be considered malignant or aggressive if they demonstrate a proclivity to recur.

The usual treatment for spinal meningiomas is resection, and complete resection can often be achieved. The dural
origin is generally cauterized and occasionally resected. Thoracic spinal roots may be sacrificed as necessary to
obtain a complete resection; cervical and lumbar nerve roots are preserved whenever possible.

Subtotally resected lesions are generally followed expectantly for regrowth. Symptomatic recurrences are generally
treated with further surgery. Radiation therapy or radiosurgery may be useful in patients with tumor regrowth that is
not amenable to further resection [34,35].

Nerve sheath tumors — Nerve sheath tumors constitute about 25 percent of tumors arising in the intradural
extramedullary space. The clinical presentation, treatment, and prognosis of these tumors is discussed separately.
(See "Intradural nerve sheath tumors".)

EXTRADURAL PRIMARY TUMORS — The most common tumors arising in the extradural space are metastases.
Several uncommon primary tumors can also arise in the extradural space.

Metastases — Metastatic tumor from any primary site can involve the epidural area and can cause epidural spinal
cord compression. The three most common primary tumors are prostate cancer, breast cancer, and lung cancer.
The clinical features and management of this complication are discussed separately. (See "Clinical features and
diagnosis of neoplastic epidural spinal cord compression, including cauda equina syndrome" and "Treatment and
prognosis of neoplastic epidural spinal cord compression, including cauda equina syndrome".)

Chordomas — Chordomas are rare bone tumors that are locally invasive, frequently recur, and are relatively
radioresistant (image 5). The tumors are remnants of the primitive notochord and may occur at the skull base (35
percent), cervical, thoracic and lumbar spine (15 percent), and sacral regions (50 percent). The presentation and
management of patients with chordoma arising in the skull base are discussed separately. (See "Chordoma and
chondrosarcoma of the skull base".)

Surgery and RT — The initial treatment of chordomas arising in the spine consists of wide local excision when
possible [36-38]. Complete resection, although desirable, is not feasible in many cases because of anatomic
constraints to surgical access and the proximity of adjacent critical normal structures. For this reason,
postoperative radiation therapy is frequently used [37,39].
The results following surgery are illustrated by a retrospective series of 138 consecutive patients treated at two
institutions over a 28 year period [36]. Lesions were in the sacrum, lumbar spine, or cervical-dorsal spine in 78, 15,
and 7 percent of cases respectively; none were in the skull base. Surgical resection was the initial therapy in 130
cases (94 percent). At a median follow-up of 12 years, the 10-year local relapse-free survival, distant relapse-free
survival, and overall survival rates were 33, 72, and 54 percent, respectively. Although surgery was supplemented
with radiation therapy in 31 percent of cases, doses were generally lower than those used in contemporary series.

For patients with inoperable lesions, newer radiation therapy techniques, including stereotactic radiosurgery and
charged particle irradiation, have been used to target the bone lesion while reducing the radiation exposure to the
surrounding nerve roots and the cauda equina [40-43].

Systemic therapy — Systemic therapy for the treatment of relapse after maximal surgery and/or radiation
therapy has focused on molecularly targeted therapies [44].

A prospective phase II study [45] and multiple observational series [46-49] have shown significant antitumor activity
with imatinib. In some instances, tumor responses have been manifested by necrosis or decreased uptake on PET
scan. Patients who have progressed after an initial response to imatinib have been reported to respond to
combinations of imatinib plus cisplatin [49] or sirolimus [47]. Other targeted agents that may have activity include
sunitinib [50] and erlotinib [51].

Older series that used cytotoxic agents alone have not reported clinically significant activity. There is only limited
experience with chemotherapy for the treatment of relapse after maximal radiation and surgery. Reported series
generally include chordomas arising in the base of skull as well as the spinal cord. The use of cytotoxic agents has
not been demonstrated to have clinically significant activity [52,53].

Sarcomas — Sarcomas arise from mesenchymal elements and may erode into the bony spine. Rarely, they can
arise directly from bony elements of the spine [54]. These lesions usually occur in younger patients, and are
treated with a multimodality approach that includes surgery, radiotherapy and chemotherapy. (See "Clinical
presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Introduction'.)

Bone sarcomas, particularly osteosarcomas, may develop in patients with polyostotic Paget disease
(pagetic sarcoma). This is a rare anaplastic malignancy with a peak incidence in the seventh and eighth
decades of life. (See "Osteosarcoma: Epidemiology, pathogenesis, clinical presentation, diagnosis, and
histology", section on 'Paget disease and other benign bone lesions'.)

Paget's osteosarcomas are usually sclerotic lesions, occasionally blastic. Treatment usually consists of
vertebrectomy, followed by chemotherapy and radiation. The prognosis of this tumor is poor.

Chondrosarcoma is a malignant tumor of cartilage in which the matrix is entirely chondroid in nature. The
presence of discrete calcified opacities is a radiographic hallmark of these lesions. Chondrosarcomas of the
spine are relatively infrequent (5 percent of all spinal tumors). They typically originate in the vertebral body
and extend into the adjacent soft tissue and spinal canal. These tumors have a predilection for the lower
thoracic and lumbar regions. Surgery is the primary treatment for chondrosarcomas. RT and chemotherapy
play a minor role and are only used in high-grade chondrosarcomas. (See "Chondrosarcoma" and
"Chordoma and chondrosarcoma of the skull base".)

Leiomyosarcoma is a malignant mesenchymal tumor composed predominantly of spindle cells that exhibit
smooth muscle differentiation. Primary leiomyosarcoma of the bone is extremely rare [55,56].

Ewing sarcoma is a small round cell tumor, which can arise in bone or soft tissue. The presentation,
management, and prognosis of the Ewing sarcoma family of tumors are discussed separately. (See "Clinical
presentation, staging, and prognostic factors of the Ewing sarcoma family of tumors".)

Lymphoma — Lymphomatous involvement of the spine is most often due to metastatic disease but occasionally
can represent primary disease. Treatment can include surgery, radiotherapy, and chemotherapy. (See "Primary
lymphoma of bone".)

Plasmacytomas and multiple myeloma — The spine can be the initial site of involvement for plasma cell
neoplasms. Although plasmacytomas can be solitary, these lesions more commonly are part of a diffuse process.
(See "Diagnosis and management of solitary plasmacytoma of bone" and "Diagnosis and management of solitary
extramedullary plasmacytoma" and "Clinical features, laboratory manifestations, and diagnosis of multiple
myeloma".)

Langerhans cell histiocytosis (eosinophilic granuloma) — Langerhans cell histiocytosis (eosinophilic

granuloma) can present at a variety of sites, including the vertebral bodies. (See "Langerhans cell histiocytosis
(eosinophilic granuloma) of bone in children".)

Benign lesions — Benign lesions can arise in the spine and must be differentiated from malignant tumors [57]:

Osteoid osteomas — Osteoid osteomas are small lesions (<2 cm) that typically arise in long bones,
although about 10 percent occur in the spine. Conservative management with salicylates is often sufficient.
(See "Overview of benign bone tumors in children and adolescents", section on 'Osteoid osteoma'.)

Osteoblastomas — Osteoblastomas are larger lesions (>2 cm) that also typically occur in young men.
Because of their size, these lesions tend to cause neurologic symptoms. Although benign, these lesions
tend to recur and generally are managed surgically. (See "Overview of benign bone tumors in children and
adolescents", section on 'Osteoblastoma'.)

Osteochondromas — Osteochondromas are benign lesions that account for less than 4 percent of spinal
tumors. These lesions consist of both healthy bone and a cartilaginous cap. Biologic behavior varies, and
surgery should be considered only for lesions producing symptoms. The most important complication is
malignant degeneration to a peripheral chondrosarcoma, the risk of which is less than 1 percent. (See
"Overview of benign bone tumors in children and adolescents", section on 'Osteochondroma and hereditary
multiple osteochondromas' and "Chondrosarcoma", section on 'Osteochondroma'.)

Chondroblastomas — Chondroblastomas are tumors arising in bone and comprised of immature cartilage;
only rarely have these been reported to arise in the spine. (See "Overview of benign bone tumors in children
and adolescents", section on 'Chondroblastoma'.)

Giant-cell tumors — Giant-cell tumors comprise 4 to 8 percent of all primary bone tumors; up to 10 percent
may arise in the spine, particularly in younger individuals. Giant-cell tumors tend to be larger, very vascular,
and have a high frequency of recurrence. (See "Giant cell tumor of bone".)

Vertebral hemangiomas — Vertebral hemangiomas are nonneoplastic lesions that are composed of thin-
walled blood vessels. Typically these are an incidental finding in the spine, and only rarely produce
symptoms [58].

Aneurysmal bone cysts — Aneurysmal bone cysts are nonneoplastic, expansile lesions, which account for
up to 15 percent of primary spine tumors [59]. These lesions tend to be locally aggressive with a substantial
potential for neurologic compromise. Thus early surgical resection and spinal stabilization is generally
required [60]. (See "Overview of benign bone tumors in children and adolescents", section on 'Aneurysmal
bone cyst'.)

SUMMARY AND RECOMMENDATIONS — Tumors affecting the spinal cord can be classified as intramedullary,
extramedullary intradural, or extradural (figure 1).

Primary intramedullary tumors — The most common intramedullary tumors include ependymomas, pilocytic
astrocytomas, and diffuse fibrillary astrocytomas. Myxopapillary ependymomas constitute a distinct subset of
ependymomas that occur almost exclusively in the conus medullaris and filum terminale of the spinal cord. (See
'Intramedullary tumors' above.)

Maximal surgical resection is the initial step in the management of patients with intramedullary spinal cord
tumors. (See 'Management of ependymomas and astrocytomas' above.)

For patients with low or intermediate grade (WHO I or II) intramedullary glial tumors in whom the initial
surgery results in a complete resection, we suggest observation rather than adjuvant radiation therapy (RT)
(Grade 2C). (See 'Ependymoma' above and 'Astrocytomas' above.)

For patients in whom complete resection has not been possible (ie, biopsy only or incomplete resection), we
suggest postoperative adjuvant RT (Grade 2C). Radiation therapy may also be useful for patients with
recurrent disease following initial resection. Rarely, observation may be a reasonable alternative for individual
patients based upon a consideration of particular details. (See 'Ependymoma' above and 'Astrocytomas'
above.)

Intradural extramedullary lesions — The most common intradural, extramedullary tumors are meningiomas and
nerve sheath tumors. The presentation and management of these tumors is discussed separately. (See
"Meningioma: Clinical presentation and diagnosis" and "Intradural nerve sheath tumors".)

Extradural tumors — The vast majority of tumors occurring in the extradural space are metastases. These tumors
are particularly important because of the risk of epidural spinal cord compression. The clinical features and
management of this complication are discussed separately. (See "Clinical features and diagnosis of neoplastic
epidural spinal cord compression, including cauda equina syndrome" and "Treatment and prognosis of neoplastic
epidural spinal cord compression, including cauda equina syndrome".)

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Topic 5227 Version 11.0

GRAPHICS

Location of spinal tumors

Spinal cord myxopapillary ependymoma

Sagittal and axial contrast enhanced MRI study of a small, cauda

equine lesion (arrow) found in a 49-year-old woman with left
buttock and leg pain. Complete resection was performed and the
diagnosis of myxopapillary ependymoma was confirmed.
Courtesy of William Welch, MD.
Pilocytic astrocytoma

Cervical spine MRI postenhancement in a 20-year-old male. Left panel: The

sagittal image demonstrates a contrast-enhancing, circumscribed lesion at
the C6-7 level (arrow). Right panel: Axial images confirm this finding
(arrows). The lesion was completely excised without causing neurological
deficit and no radiation was administered.
Courtesy of William Welch, MD.
Intramedullary metastatic cancer

Lumbar MRI scan in a 57-year-old man with known squamous cell

carcinoma of the ethmoid sinus with metastasis to the skull base.
The sagittal enhanced images demonstrate a large, intradural
metastasis at the L1 level (arrow). Surgical pathology was
consistent with metastatic squamous cell carcinoma.
Courtesy of William Welch, MD.
Thoracic spine meningioma

Contrast-enhanced thoracic MRI scans in a 75-year-old woman with

progressive paraparesis due to a mid-thoracic spinal meningioma. Left panel:
The sagittal study reveals a dural-based lesion at T7-8. Right panel: The
axial image shows the ventrally-located lesion (arrow) causing spinal cord
compression.
Courtesy of William Welch, MD.
Chordoma

MRI and CT studies on a 70 year-old woman with chordoma. The

patient had undergone subtotal resection of the lesion and
received postoperative fractionated radiation therapy. Top panel:
A sagittal, contrast-enhanced MRI demonstrates recurrent
chordoma in the sacral region (arrow). Bottom panel: A contrast-
enhanced axial CT scan also shows recurrent tumor (arrow). The
patient underwent stereotactic radiosurgery but the tumor
progressed.
Courtesy of William Welch, MD.