N E U R O S U R G E RY A RT I C L E

Intramedullary Spinal
Cord Tumours:
Neil R Malhotra
is a Neurosurgeon at the University of
Pennsylvania (USA). His primary research interests
diagnosis, treatment, and outcomes
focus on restorative approaches and tissue engi-
neering to develop treatments to restore native
tissue mechanics of the spine while delivering
therapeutic agents and supporting tissue regenera- ntramedullary tumours are lesions that usual- drome, hemiparisis ipsilateral to the lesion with
tion. Dr Malhotra provides expertise in the care of
a broad range of brain and spinal neurological dis-
orders. He takes particular interest in the develop-
ment of less invasive techniques for spinal recon-
struction and tumour resection.
I ly arise directly from the neural tissue of the
spinal cord. They are unique amongst all
spinal column masses in that they require inci-
loss of pain and temperature sensation on the
contralateral side, has also been reported in
patients with IMSCT. Tumours of the cervical or
sion of the spinal cord for surgical access. thoracic spine may lead to lower extremity
Intramedullary tumours of the spine make up 2- spasticity. Urinary retention and incontinence
4% of all central nervous system tumours and are more common in lower cord tumours,
are a rare cause of spinal cord dysfunction.1 although urinary symptoms may be late seque-
Tumours of the spinal column may be classified lae of any lesion. Faecal incontinence is much
by location as extradural, intradural, or less common. It is important to note that all
intramedullary. Amongst all masses of the patients with signs of new or unexplained
spinal column, intramedullary tumours are the myelopathy such as spasticity, hyperreflexia,
least common to be encountered in the general incoordination, or gait disturbance should have
Deb Bhowmick public. further imaging of the spine and appropriate
is a seventh year resident at the University of Their rare incidence commonly results in mis- neurological follow-up.
Pennsylvania Department of Neurosurgery with diagnosis and improper diagnostic workup,
an interest in spinal tumours. resulting in delayed diagnosis. More common Differential diagnosis
clinical entities such as arthritic spinal myelopa- The differential diagnosis of the most common
thy, multiple sclerosis, or even aortic dissection presenting signs and symptoms include IMSCT,
can be confused with intramedullary tumours as intradural extramedullary spinal tumours,
they may have similar clinical and radiographic epidural spinal tumours, myelopathy due to
presentations. degenerative disease, cord infarct, vascular
Deferred diagnosis and treatment can lead to lesions such as spinal arteriovenous malforma-
progressive paralysis, urinary and faecal inconti- tions and dural arterio-venous fistulae, the
nence, as well as reduced survival. Imaging and inflammatory processes such as multiple sclero-
surgical technologies were inadequate to diag- sis, transverse myelitis and sarcoid. Perhaps most
Douglas Hardesty nose or treat these tumours without serious mor- challenging for the general practitioner is differ-
is a fourth-year MD candidate at the University
of Pennsylvania School of Medicine (USA). His bidity in the past. Today, convenient high resolu- entiating between degenerative spinal canal
research interests are primarily in central nervous tion MRI as well as improved surgical adjuvants stenosis, a relatively common condition, and
system tumour signaling mechanisms. He is pur- and techniques have allowed for significantly IMSCT (or other spinal tumours), which are rela-
suing a career in academic neurosurgery.
improved resection and overall neurological out- tively rare. The medical history is often helpful, as
come. Most patients can now undergo definitive patients with degenerative disease tend to have
diagnosis and treatment without significant long- years of waxing and waning pain, frequently
term loss of spinal cord function. Thus, awareness accompanied by radicular symptoms. Patients
of this clinical entity and early diagnosis and with lumbar stenosis often complain of postural
treatment is paramount to avoid disability. back or radicular pain, worst in torso extension
(i.e. ascending stairs) and less painful in torso
Clinical Presentation flexion (i.e. pushing a shopping cart).
Intramedullary spinal cord tumours (IMSCT) Neurogenic claudication (radicular pain and
have a myriad of presenting signs and symp- paraesthesias or numbness with ambulation)
Peter Whitfield toms, making a simple diagnositic algorithm dif- due to lumbar spinal stenosis is characteristical-
is a Consultant Neurosurgeon at the South West
Neurosurgery Centre, Plymouth. His clinical inter-
ficult. The temporal course of these lesions is ly relieved by several minutes of sitting, while the
ests are wide, including head injury, stereotactic widely disparate, with occasional patients pre- radicular pain due to IMSCT is usually not pre-
radiosurgery, image guided tumour surgery, neu- senting with acute neurologic deficits and oth- cipitated by walking nor relieved by rest.
rovascular surgery, lumbar and cervical microdis-
ers with a protracted course. Most series
cectomy. He is an examiner for the Intercollegiate
MRCS and a member of the SAC in Neurosurgery. demonstrate that the most common presenting Imaging
complaint is either local dull pain or radicular T1- and T2-weighted MRI with and without
Correspondence to: pain, often associated with some degree of gadolinium is the imaging modality of choice
Neil R. Malhotra MD, Assistant Professor,
Department of Neurological Surgery,
lower extremity numbness.2,3 The finding of for suspected IMSCT. MRI allows the clinician to
University of Pennsylvania, local back pain when lying flat in bed (noctur- narrow the broad differential diagnosis listed
Penn Neurological Institute, nal pain) is highly suggestive of tumour, espe- above, and interpretation by an experienced
Washington Square West Bldg,
cially if this pain wakes the patient from sleep; neuroradiologist is helpful. Ependymomas and
235 S. Eighth St, Philadelphia, PA 19106
Email: [email protected] however, these alerting symptoms are often astrocytomas have similar imaging characteris-
absent. Motor weakness is also common, tics and a definitive diagnosis is only made
although it usually presents later than pain or intraoperatively with a tissue sample. Thus, the
sensory disturbances. The Brown-Sequard syn- role of MRI is not to distinguish between differ-

ACNR > VOLUME 10 NUMBER 4 > SEPTEMBER/OCTOBER 2010 > 21

N E U R O S U R G E RY A RT I C L E

Table 1: Differential diagnosis of spinal cord dysfunction or clinical myelopathy Table 2: Symptoms and signs
with prevalence of the disease in the general population. Note: not all persons associated with intramedullary
will manifest symptoms or require medical attention in some disease states. spinal cord tumours.
Symptoms
Diagnosis Prevalence of Disease
Gait Imbalance
Cervicothoracic spondylosis 13%
Upper or lower extremity weakness
B12 deficiency 1%
Upper or lower extremity numbness
Normal pressure hydrocephalus 0.5%
Urinary incontinence
Multiple sclerosis 0.14%
Nocturnal back pain
Syringomyelia 0.008%
Hand incoordination
ALS 0.002%
Burning dysaesthesias
Extrinsic tumours of the spine 0.0003%
Sudden paraparesis or quadraparesis
IMSCT 0.00001%
Signs
Spinal cord infarction unknown
Spasticity
Hyperreflexia
Upward Babinski sign
Objective bilateral weakness
Sensory level
Positive Hoffman’s sign

Treatment
Referral to a neurosurgical specialist for treat-
ment and management of intramedullary
masses is very important. This is because, with
very few exceptions, all newly diagnosed
intramedullary masses require total or sub-
total resection or biopsy for tissue diagnosis.
Additionally, the surgeon may be able to pro-
vide adequate decompression of the spinal
cord to avert progression of neurological
compromise. It is also possible that a com-
plete surgical resection, if possible, may result
in the definitive treatment of many of the
tumours of the spinal cord known to be
pathologically benign.
Surgery for the resection of intramedullary
tumours involves the exposure and decom-
pression of the spinal cord, usually through a
multilevel laminectomy followed by a midline
dorsal dural opening. Localisation of the
laminectomy can be performed using spinal
needles and spinal imaging (image intensifi-
er) in the operating theatre. Opening of the
Figure 1. An ependymoma of the cervical spine on MRI T2 image (left) and T1 with contrast (right). Note the associated syrinx
seen on T2, contrast enhancing mass on T1, and overall expansion of the cord. spinal cord in order to access the spinal
tumour is commonly done through a longitu-
dinal, midline incision. This is done to avoid
ent IMSCT types, but to rule out lesions such nature of most IMSCT. Associated cysts are transection of the white matter tracts of the
as infarct, and auto-immune and inflamma- also common with IMSCT, and may appear dorsal columns and avoid disturbing motor
tory diseases which will not benefit from sur- similar to tumour on T1 and T2-weighted and cerebellar long tracts found laterally and
gical intervention. Non-IMSCT lesions tend images but can be differentiated from tumour ventrally in the spinal cord. Intraoperative
to have little or no cord enlargement or by their lack of gadolinium enhancement.4 ultrasound is commonly employed prior to
oedema, thus differentiating them from IMSCT at any level may be associated with a myelotomy to accurately localise the spinal
IMSCT. If the patient is relatively stable and syrinx; these are especially common in IMSCT cord lesion and minimise the extent of the
the MRI is equivocal, repeat imaging after of the cervical spine. Once an IMSCT is incision. 5
one month should show decreased oedema demonstrated on MRI, prompt referral to a Modern surgical techniques and adjuncts
and mass effect in acute auto-immune neurosurgeon is warranted. Patients in which employed during surgery have led to
lesions.4 MRI is contraindicated may benefit from CT improved neurological outcomes and sur-
Sequences in the sagittal and axial planes myelography, although this modality is much vival from intramedullary tumours. The
are most useful in pre-operative planning. less useful than MRI. Plain films do not have a employment of the operative microscope as
Enhancement on T1-weighted gadolinium significant role in the evaluation of suspected well as ultrasonic aspiration devices during
sequences is common, despite the low-grade IMSCT. the exposure and resection of the tumour

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N E U R O S U R G E RY A RT I C L E

have resulted in an overall neurological mor-

bidity rate of 34% for this type of surgery in
current cohorts with most patients improving
within 1 month of surgery.6 Active neu-
romonitoring during the surgical case has
also been a technical advance employed
during resection. Combined use of
somatosensory evoked potentials (SSEPs)
and motor evoked potentials (MEPs) has
been shown to reduce surgical morbidity by
providing feedback to the surgeon when
resection manoeuvres or effects of anaesthe-
sia during the surgery are putting the spinal
cord viability at risk.7
Currently, most neurosurgeons tailor their
resections depending on visualised anatomic
planes, neuromonitoring changes, and results of
intraoperative histology. The presence of a visu-
alised plane between the tumour tissue and the
tissue of the spinal cord greatly improves over-
all resection, neurological outcome, and overall
survival.6 The presence of a syrinx that is con-
tinuous with the tumour plane also improves
overall neurological outcome.6,8 The loss of
greater than 50% of MEP signals during the case
Figure 2. A fibrillary astrocytoma of the cervical spine on T2 (left) and T1 with contrast (right). Note the indistinct cord oede- is sensitive for the appearance of significant
ma, expansion and partial contrast enhancement.
motor deficits post-operatively.7,9 Another neu-
romontoring adjuvant, D-wave monitoring, is
often employed during surgery and has been
shown to correlate with neurological outcome
after surgery when used to tailor resections.7
Non-surgical treatments for intramedullary
tumours are largely relegated to patients with
diffuse inoperable tumours, those with known
incomplete resection, recurrent tumours, or
those who could not tolerate surgery or have
such a poor prognosis from their primary dis-
ease process that surgery would be an ineffec-
tive intervention. Historically, external beam
radiation has been employed as a treatment
for these patients. There exists favourable evi-
dence that radiation likely increases the pro-
gression free survival of patients with low
grade astrocytomas and ependymomas after
partial resection.10,11 However, evidence is limit-
ed on the overall effectiveness of radiation for
patients with new progression or malignant
pathologies. Although there is an expectation
that chemotherapeutic agents may be effec-
tive in the treatment of malignant astrocy-
tomas of the spinal cord, there is no evidence
Figure 3. A haemangioblastoma of the thoracic spine on T1 with contrast in sagittal (left) and axial sections (right). Note the that they improve overall survival.
enhancing tumour nodule with associated cyst.

Table 3: Radiographic differential diagnosis of an intramedullary lesion.

Diagnosis/Characteristics Cord Contrast enhancement Well circumscribed Heterogeneous Signal Cord oedema
Expansion

Ependymoma + + + - -/+
Astrocytoma + + -/+ - -/+
Haemangioblastoma + + + - +
Cavernoma + + + + -/+
Multiple sclerosis -/+ -/+ -/+ - +
Transverse myelitis -/+ -/+ - - +
Spinal cord infarct -/+ - + - +
AV fistula - - - - +

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N E U R O S U R G E RY A RT I C L E

Outcomes ily correlate with progression-free survival in with fusion has been favourably applied with
Neurological outcome from surgery is highly low grade astrocytomas, however, a growing improved prevention of postoperative spinal
correlated with preoperative deficits. Patients body of evidence suggests that an increased deformities in children.19
with fewer preoperative symptoms are more extent of resection is beneficial to overall Currently, outcome-modifying treatments
likely to have good postoperative neurological neurological outcome in this group of for malignant astrocytomas of the spinal cord
outcomes.6,12 Factors predictive of worse post- patients.17 Surgeons are commonly less do not exist. Patients are empirically treated
operative neurological outcomes include tho- aggressive with resections if intraoperative with postoperative radiation with or without
racic location, advanced age and the presence histology during the case indicates a diagno- chemotherapy with universally poor results.
of urinary symptoms.6,12 Up to one third of all sis of astrocytoma and are conversely more Aggressive resection, including cordotomy, has
patients have a significant, acute deterioration aggressive for ependymomas and heman- not yielded any benefit to outcome and most
in neurological function after surgery, with gioblastomas. die of complications of paralysis or progres-
nearly half returning to their preoperative sta- Given the greatly improved survival of many sion of disease.
tus within a month of surgery.6 of the intramedullary tumour patients, the addi-
The most important predictor for tumour tion of spinal fusion techniques with internal Conclusion
recurrence and survival is pathology. rod or plate fixation has also been investigated Intramedullary spinal cord tumours are rare
Malignant astrocytomas of the spinal cord to prevent long-term spinal deformity from the but important clinical entities that now can be
have an overall recurrence rate of greater surgery. The presence of preoperative scolio- easily diagnosed and often effectively treated.
than 95%, with outcome unaffected by extent sis, syrinx, long-standing neurological deficit, or Importance should be given to recognising
of surgery.13,14 Complete resection of ependy- cervicothoracic junction location has been clinical and radiographic findings that are
momas and haemangioblastomas, however, correlated with the development of post-opera- associated with these tumours. Surgical
carry a very favourable outcome, with recur- tive spinal deformities that can be functionally advances have now made many of these
rence rates less than 10% over a ten year peri- limiting.18 Thus the use of osteoplastic lamino- tumours treatable with acceptable long-term
od. 15,16 Extent of resection does not necessar- plasty or preemptive internal spinal fixation neurological outcomes. l

REFERENCES

1. Kane PJ, el-Mahdy W, Singh A, Powell MP, Crockard, HA. 8. Samii M and Klekamp J. Surgical results of 100 14. Constantini S, Miller DC, Allen JC, Rorke LB, Freed D,
Spinal intradural tumours: Part II--Intramedullary. Br J intramedullary tumors in relation to accompanying Epstein FJ. Radical excision of intramedullary spinal cord
Neurosurg, 1999:13(6);558-63. syringomyelia. Neurosurgery, 1994;35(5):865-73; discus- tumors: surgical morbidity and long-term follow-up evalua-
2. Houten JK and Cooper PR. Spinal cord astrocytomas: presen- sion 873. tion in 164 children and young adults. J Neurosurg,
tation, management and outcome. J Neurooncol, 2000;93(2 Suppl):183-93.
9. Quinones-Hinojosa A, Lyon R, Zada G, et al. Changes in
2000;47(3):219-24.
transcranial motor evoked potentials during intramedullary 15. Binning M, Klimo P Jr, Gluf W, Goumnerova L. Spinal
3. Schwartz TH and McCormick PC. Intramedullary ependymo- spinal cord tumor resection correlate with postoperative tumors in children. Neurosurg Clin N Am,
mas: clinical presentation, surgical treatment strategies and motor function. Neurosurgery, 2005;56(5):982-93; discus- 2007;18(4):631-58.
prognosis. J Neurooncol, 2000;47(3):211-8. sion 982-93.
4. Waldron JS and Cha S. Radiographic features of 16. Epstein FJ. Spinal cord tumors in children. J Neurosurg,
intramedullary spinal cord tumors. Neurosurg Clin N Am, 10. Chun HC, Schmidt-Ullrich RK, Wolfson A, Tercilla OF, 1995;82(3):516-7.
2006;17(1):13-9. Sagerman RH, King GA. External beam radiotherapy for pri-
mary spinal cord tumors. J Neurooncol, 1990;9(3):211-7. 17. Jallo GI, Danish S, Velasquez L, Epstein F. Intramedullary
5. Epstein FJ, Farmer JP and Schneider SJ. Intraoperative ultra- low-grade astrocytomas: long-term outcome following radical
sonography: an important surgical adjunct for intramedullary 11. Linstadt DE, Wara WM, Leibel SA, Gutin PH, Wilson CB, surgery. J Neurooncol, 2001;53(1):61-6.
tumors. J Neurosurg, 1991;74(5):729-33. Sheline GE. Postoperative radiotherapy of primary spinal
cord tumors. Int J Radiat Oncol Biol Phys, 18. McGirt MJ, Chaichana KL, Atiba A, Bydon A, Witham TF,
6. Garces-Ambrossi GL, McGirt MJ, Mehta VA et al. Factors
1989;16(6):1397-403. Yao KC, Jallo GI. Incidence of spinal deformity after resection
associated with progression-free survival and long-term neuro-
of intramedullary spinal cord tumors in children who under-
logical outcome after resection of intramedullary spinal cord
12. McGirt MJ, Chaichana KL, Atiba A, Attenello F, Yao KC, went laminectomy compared with laminoplasty. J Neurosurg
tumors: analysis of 101 consecutive cases. J Neurosurg Spine,
Jallo GI. Resection of intramedullary spinal cord tumors in Pediatr, 2008;1(1):57-62.
2009;11(5):591-9.
children: assessment of long-term motor and sensory deficits. J
7. Sala F, Palandri G, Basso E, et al. Motor evoked potential Neurosurg Pediatr, 2008;1(1):63-7. 19. Simon SL, et al. Efficacy of spinal instrumentation and fusion
monitoring improves outcome after surgery for intramedullary in the prevention of postlaminectomy spinal deformity in chil-
spinal cord tumors: a historical control study. Neurosurgery, 13. Cohen AR, Wisoff JH, Allen JC, Epstein F. Malignant astro- dren with intramedullary spinal cord tumors. J Pediatr
2006;58(6):1129-43; discussion 1129-43. cytomas of the spinal cord. J Neurosurg, 1989;70(1):50-4. Orthop, 2008;28(2):244-9.

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26 > ACNR > VOLUME 10 NUMBER 4 > SEPTEMBER/OCTOBER 2010