Summary: A 29-year-old pregnant woman presented to her primary care physician
with mild right leg weakness and numbness 6 weeks before delivery. The
symptoms were attributed to her pregnancy and improved somewhat after
delivery, although she continued to have minor symptoms. Approximately 6
months later, her symptoms worsened abruptly. She presented to a
neurologist with bilateral lower extremity numbness and impaired
vibratory sensation and proprioception. A magnetic resonance imaging
(MRI) examination of the lumbar spine was performed (Figure 1), which
prompted further MRI of the entire neural axis (Figure 2).
Anaplastic spinal cord ependymoma
Sagittal T1-weighted and T2-weighted MR images of the lumbar spine
demonstrate an enhancing intradural intramedullary mass extending from
T10 to the conus medullaris (Figure 1). Sagittal T1 pre- and
postcontrast (Figure 2) MR images reveal enhancement throughout the
subarachnoid space of the lumbar and thoracic spine extending superiorly
to the level of the cervical spine (not shown)and into the basilar
cisterns (Figure 2).
Some 90% to 95% of spinal cord neoplasms are of glial origin, with
ependymomas and astrocytomas accounting for up to 70% all intramedullary
neoplasms. Astrocytomas are more common in children, while ependymomas
are more common in adults. The mean age at presentation for ependymoma
is 38.8 years.1 However, they remain a fairly rare entity, with approximately 227 diagnosed in theUnited States each year.2
most often present with neck or back pain. Sensory deficits, weakness,
and bowel and bladder complaints are also common.3 The mean
time from presentation to diagnosis is approximately 3 years. Generally,
patients with minor preoperative deficits have better outcomes.1
Treatment usually consists of gross total resection. The role of
radiation therapy (RT) is controversial, although it is usually given in
cases of subtotal resection or recurrence.2 Opponents of RT
argue that reoperation becomes very difficult after therapy, and that
radiation myelopathy could worsen neurological deficits.3 The 5-year survival rate is approximately 82%.1
ependymomas demonstrate intense enhancement with intravenous contrast
on computed tomography (CT). On T1-weightedMR imaging, ependymomas are
isointense to hypointense with respect to the spinal cord, although they
may rarely be hyperintense inthe setting of hemorrhage. Ependymomas are
isointense to hyperintense on T2-weighted images. Approximately
one-third of ependymomas will have a rim of hypointensity at the poles
on T2-weighted images. This is known as the “cap sign,” which is
believed to be secondary to hemorrhage. Ependymomas will also enhance on
MR after contrast administration.1
Three types of
cysts are associated with ependymomas. Intratumoral cysts are located
within the tumor and are believed to be secondary to degeneration. They
are of variable signal intensity, demonstrate peripheral enhancement,
and should be surgically excised. Rostral and caudal cysts are the
second type and occur above and below the tumor. They are reactive in
nature, demonstrate no enhancement,and do not require excision. The
third cyst type, dilation of the central canal, is likely reactive in
etiology as well, from partial canal obstruction by the tumor. Again, no
enhancement is seen, and the dilation resolves after tumor excision.4
6 main histologic types of ependymoma are cellular, papillary,
tancytic, clear cell, myxopapillary, and melanotic. Cellular is the most
common type, and melanotic is the least common. The distinguishing
histologic characteristic is perivascular pseudorosette formation. Most
ependymomas are classified as either World Health Organization grade I
or II, while malignant (grade III) types are rare.1,4
Ependymoma is the most common intramedullary spinal cord neoplasm of
glial origin in adults, although its incidence remains relatively rare.
Ependymomas are treated with gross total resection, and MRI
characteristics of associated cysts are important for surgical planning.
The role of adjuvant therapy remains controversial.
- Koeller KK, Rosenblum RS, Morrison AL. Neoplasms of the spinal cord and filum terminale: Radiologic-pathologic correlation. Radiographics. 2000;20:1721-1749.
- Shors SM, Jones TA, Jhaveri MD, Huckman MS. Myxopapillary ependymoma of the sacrum. Radiographics. 2006;26:S111-S116.
- Hanbali F, Fourney DR, Marmor E, et al. Spinal cord ependymoma: Radical surgical resection and outcome. Neurosurgery. 2002;51:1162-1174.
- Kahan H, Sklar EML, Post JD, Bruce JH. MR characteristics of histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroadiol. 1996;17:143-150.