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Technical aspects of imaging choice for diagnosing and character- The MRI protocol typically includes
spine tumors izing pediatric spine tumors. With its unenhanced sagittal T1-, sagittal and
Magnetic resonance imaging has multi-planar imaging capability, supe- axial T2-weighted images, and con-
emerged as the imaging modality of rior contrast resolution, and inherent high trast-enhanced T1-weighted images in
signal-to-noise ratio, MRI is best suited to the sagittal and axial planes. Coronal
Dr. Shekdar and Dr. Schwartz are neu- provide information regarding the exact images may be particularly useful for
roradiologists at the Children’s Hospi- location, extent and morphological fea- intradural/extramedullary tumors and
tal of Philadelphia, Philadelphia, PA. tures of these tumors. This is crucial in in evaluating tumors with para-spinal
Dr. Shekdar is an Assistant Professor providing a precise diagnosis, or at least extension. Diffusion-weighted imag-
of Clinical Radiology and Dr. Schwartz
is an Associate Professor of Radiology narrowing the differential diagnosis. ing may be particularly useful in char-
at the Perelman School of Medicine at MRI does not involve ionizing radiation, acterizing high-grade neoplasms and
the University of Pennsylvania, which is particularly important with re- evaluating dermal inclusion cyst/epi-
Philadel- phia, PA. spect to imaging the pediatric population. dermoid tumors. Ongoing work in more
Intramedullary tumors
Astrocytoma
Intramedullary tumors comprise ap-
proximately 10 percent of all primary
CNS tumors, but 25 percent of those in
the pediatric population.1,3 Astrocytoma
is the most common intramedullary
tumor in this group, accounting for up
to 60 percent.2,4 Controversy remains
regarding the second-most common
type of intramedullary tumor—whether
it is ganglioglioma or ependymoma.
Older literature favors ependymoma,
while most of the more recent pediatric
literature argues for ganglioglioma, ac-
counting for up to 15 percent.1,3
Ependymomas of the spinal cord are
usually seen in the setting of neurofi-
D E bromatosis type 2, but are otherwise
uncommon in children. The more com-
mon myxo-papillary subtype is usu-
ally seen in adolescents and is typically
intradural/extramedullary in location.5
Other intramedullary cord tumors seen
in children include atypical teratoid-
rhabdoid tumors, hemangioblastomas
FIGURE 1. Sagittal T2 (A) and contrast-enhanced T1 of the cervical spine (B), and thoracic and, rarely, intramedullary metasta-
spine (C) and axial T2 (D) and contrast-enhanced T1 (E) of the cervical spine show abnor-
ses.1,3,6
mal expansion of the spinal cord due to a heterogeneous, intramedullary astrocytoma with
enhancing solid components (arrowheads) and non-enhancing cystic/necrotic components The astrocytoma originates from the
(white arrows). Note the non-tumor syrinx inferior to the enhancing astrocytoma (white star) astrocyte cells of the spinal cord. The
and abnormal T2 hyper-intense signal in the upper cervical spinal cord (black star) due to peri- most common histological subtype is pi-
tumoral edema. locytic astrocytoma (WHO grade I) fol-
advanced MRI techniques, such as dif- Clinical presentation of spine tumors lowed by fibrillary astrocytoma (WHO
fusion tensor imaging, promises to be a Most spinal cord tumors in children grade II).1,5 When increased cellularity,
useful tool in characterization of pediat- grow slowly. The symptoms may be mitosis, and nuclear atypia are present,
ric spinal cord tumors. vague and even misleading; hence, in it is classified as anaplastic astrocytoma
Computed tomography (CT) contin- most cases there is a significant delay in (WHO grade III). Rarely, glioblastoma
ues to have a role in assessing osseous diagnosis. Some common clinical fea- can occur in the spinal cord (WHO
spinal tumors and detecting calcifica- tures associated with spinal cord tumors grade IV). Multiple low-grade astrocy-
tion/mineralization. Judicious use of include pain, mild motor weakness, tomas may be seen in the spinal cord in
CT can be a valuable adjunct to MRI in progressive scoliosis and gait distur- the setting of neurofibromatosis type 1.
pediatric spinal tumors. bances. There may be a history of re- Astrocytomas usually affect children
The clinical presentation of spinal mission and exacerbation of symptoms between 1 and 5 years of age, with the
cord tumors may be very nonspecific, thought to be secondary to the varying most common astrocytoma being the
with pain or progressive scoliosis. degrees of peri-tumoral cord edema. pilocytic variety.2,5 Fibrillary astrocy-
Hence, many children may initially be Tumors located in the upper cervical toma is more common in older children,
imaged with conventional radiography. spinal cord or at the cranio-cervical usually above 10 years of age.
A B A
FIGURE 6. Two cases of intramedullary spinal cord ganglioglioma. Contrast-enhanced sagit- FIGURE 7. Contrast-enhanced sagittal (A)
tal T1 (A) demonstrating a ganglioglioma with multiple enhancing nodules scattered within the and axial (B) T1 images showing an eccen-
lower spinal cord and extending into the proximal cauda equina (white arrows). (B) Composed trically located enhancing, intramedullary AT/
T2 sagittal of the entire spine showing an extensive ganglioglioma involving the entire cervical RT of the upper cervical spinal cord (black
and upper thoracic spinal cord with large cystic components (black stars), several of which star) with exophytic extension (white arrow).
demonstrate internal blood/fluid-fluid levels (thick white arrow).
these may be congenital.1 Most ATRTs enhancement is commonly seen (Figure multiple hemangioblastomas may be
occur in the brain, but they may also in- 7). CSF dissemination is common; there- present (Figure 8).5 A majority of he-
volve the spinal cord. Here they may be fore, the entire craniospinal axis should mangioblastomas are intramedullary,
either intramedullary or extramedullary be imaged. The prognosis is uniformly but they may occur within the intradural
in location.1,14 They are aggressive tu- dismal due to the high tumor grade. CSF space, or even have an extradural local-
mors (WHO grade IV) that commonly dissemination and neurological dysfunc- ization. Intramedullary hemangioblasto-
infiltrate the spinal cord and surround- tion are usually noted at presentation. mas are peripheral in location, typically
ing structures. arising from the pial surface of the spi-
Many of these tumors are very large Hemangioblastoma nal cord.4 On MRI, hemangioblastomas
at presentation, showing aggressive fea- Hemangioblastomas are benign are seen as well-circumscribed nodular
tures. On imaging, ATRTs are usually (WHO grade I) but highly vascular, masses. The T1 signal may be variable
heterogeneous, with solid and cystic/ capillary-rich neoplasms. They account due to the presence of blood products,
necrotic components, and commonly for fewer than 10 percent of all spinal but they are usually T1 hypo-intense,
demonstrate extensive hemorrhage. The tumors and are rare in children.4,5 They with hyperintensity on T2 and intense
solid components demonstrate hypo- can occur sporadically, but are most enhancement following contrast admin-
intense signal on T1 and hyperinten- commonly are seen in children with von istration (Figure 8). There may be vary-
sity on T2.14 Heterogeneous contrast Hippel-Lindau (VHL) syndrome, where ing degrees of surrounding cord edema.
A B C
FIGURE 8. Contrast-enhanced T1-weighted sagittal (A & B) and axial (C & D) images in a patient with VHL syndrome with multiple scattered
enhancing nodules within the cord adjacent to the pial surface (arrows). Also note the tiny enhancing hemangioblastomas within the superior
and inferior aspect of the cerebellum (arrowheads).
In the sporadic cases with single lesions include pain, mild motor weakness, based on the location of the mass, fea-
the differential diagnostic considerations progressive scoliosis and gait distur- ture spinal-cord or nerve-root compres-
may include cavernous malformation, bances. There may be a history of re- sion. The most frequently encountered
arteriovenous malformation, or other mission and exacerbation of symptoms intradural/extramedullary spinal tumors
hypervascular spinal cord neoplasms. thought to be secondary to the varying in the pediatric age group are metastases
On angiography, hemangioblastoma degrees of peri-tumoral cord edema. from primary intracranial tumors.
is described to have a very prominent Tumors located in the upper cervical Other intradural/extramedullary
vascular blush with a feeding artery spinal cord or at the cranio-cervical spine tumors in children include the
and draining vein.1 The treatment of he- junction may present with torticollis myxo-papillary subgroup of ependy-
mangioblastoma is surgical resection. and occasionally with lower cranial momas, nerve-sheath tumors, such as
However, due to their extensive vascu- nerve palsies. Rarely, hydrocephalus schwannoma and neurofibroma;
larity, endovascular embolization often and/or raised intracranial pressure can congenital or dysontogenetic masses
precedes surgery to minimize blood be a manifestation due to altered CSF such as the dermal inclusion cyst (der-
loss.15 Other treatment modalities, such dynamics produced by the spinal tumor. moid and epidermoid cyst); neuren-
as gamma knife radiosurgery, are also teric cysts, and arachnoid cysts. Other
being utilized for the treatment of those Intradural/extramedullary tumors tumors that may also be found in the
hemangioblastomas that are not amena- Tumors in this location characteristi- intradural/extramedullary location in-
ble to surgical resection.16 cally displace and compress the spinal clude ATRTs, meningiomas and para-
cord and expand the ipsilateral thecal gangliomas.12
Clinical presentation sac. The interface formed between the le-
Most spinal cord tumors in children sion and the adjacent spinal cord results Metastases
grow slowly. The symptoms may be in the so-called “meniscus sign.”17 These The most common spinal metastases
vague and even misleading; hence, in lesions can be located in the neural fo- are from posterior fossa tumors, par-
most cases there is a significant delay in ramina, the filum terminale or the cauda ticularly medulloblastoma, followed
diagnosis. Some common clinical fea- equina nerve roots. These spinal tumors by ependymoma, among other tumors.
tures associated with spinal cord tumors most commonly present with pain, and, Metastases are usually lepto-meningeal
A B C D E
FIGURE 10. Well-demarcated ependymoma at the tip of the conus medullaris, demonstrating T2
hyper-intensity (A,C) and homogeneous enhancement on contrast-enhanced T1 sagittal & axial
(B,D). Intra-operative image of the ependymoma at surgery (E). (Courtesy Leslie Sutton, MD, CHOP)
A B C
A B C
A B C D
FIGURE 13. Ovoid mass lesion of the filum terminale (black star) which is mildly hyper-intense on sagittal T2 (A) and iso-intense on sagittal T1
(B). Note the T1 hyperintense fatty infiltration on sagittal (B) and coronal (C) T1 images superior and inferior to the dermoid (white arrows). Intra-
operative image (D) (Courtesy Leslie Sutton, MD,CHOP).
on T2-weighted images, with avid con- region, producing the characteristic moid tumors account for 10 percent of
trast enhancement. When the imaging “dumb-bell” configuration.6, 17 all spinal tumors in children. Structur-
plane is perpendicular to the long axis ally the dermoid has all the three dermal
of the neurofibroma, on T2-weighted Dysontogenetic masses layers and contains dermal appendages;
or short tau inversion recovery (STIR) This group includes non-neoplastic eg, hair, and sebaceous glands. Unlike
images, the typical “target” appearance masses of congenital origin, such as the dermoid, epidermoid has only two
may be noted.21Solitary schwannomas dermal inclusion cysts (dermoids and dermal layers and does not contain skin
as well as neurofibromas can extend via epidermoids), neurenteric cysts, and appendages. Both dermoids and epider-
the neural foramen into the para-spinal arachnoid cysts. Dermoid and epider- moids can have cystic structures and
A B C D E
FIGURE 14. Ovoid mass expanding the thoracic spinal cord, which is T2 hyperintense (A), T1 hypo-intense with a thin rim of contrast enhance-
ment (B), and exhibiting restricted diffusion (b=1000, C and ADC map, D) consistent with an epidermoid inclusion cyst. Intraoperative image (E).
(Courtesy Leslie Sutton, MD, CHOP)
A A B C
FIGURE 16. Sagittal STIR (A), contrast-enhanced T1 sagittal (B) and coronal (C) images
demonstrate a T9 vertebra plana (white arrows) from eosinophilic granuloma, with intact adja-
cent endplates (white stars).
signal characteristics of the dermoid surgical intervention, such as repair of
can vary depending on the composi- spinal dysraphism. Epidermoids display
tion of the cystic content and amount of signal intensity similar to that of CSF
solid tissue. The cystic content on MR on T1- and T2-weighted images. Dif-
may demonstrate intrinsic high T1 sig- fusion-weighted imaging is extremely
FIGURE 15. Axial bone window CT section nal and intermediate-to-low T2 signal. helpful in distinguishing an epidermoid
through the C6 vertebra (A) and sagittal ref-
Solid components of the dermoids are from arachnoid cysts due to the char-
ormation (B) show an expansile, lytic lesion
(white arrow) with sclerotic nidus (arrow- iso-to hypo-intense relative to the spi- acteristic restricted diffusion within an
head), typical of osteoid osteoma. nal cord on T1-weighted images and epidermoid.18 Similar to dermoid, epi-
hyper-intense on T2-weighted images. dermoid also demonstrates lack of con-
fluid debris with varying concentrations Typically, dermoids will not enhance trast enhancement, unless secondarily
of keratin.18 Dermoids are commonly with contrast unless secondarily in- infected (Figure 14).
associated with dermal sinus tracts. fected (Figure 13).18Epidermoids may
Complications such as infection from be primary or, as is more common in Extradural tumors
the dermal sinus tract, meningitis, and children, iatrogenic secondary to inad- Extradural tumors account for two-
abscess formation may occur. The MR vertent inclusion of skin debris during thirds of all spinal tumors in children,
and is known by other names, such as diffusion. Large masses can cause ef- 10. Hamburger C, Buttner A, Weis S. Gan-
glioglioma of the spinal cord: Report of two rare
chloroma, myeloid sarcoma, and extra facement of the thecal sac and can dis- cases and review of the literature. Neurosurgery.
medullary myeloid tumor. GS is the place and compress the spinal cord. 1997;41:1410-1415; discussion 1415-1416.
most common extra-osseous spinal Detailed discussion of all tumors in this 11. Patel U, Pinto RS, Miller DC, et al. MR of spi-
nal cord ganglioglioma. AJNR J Am Neuroradiol.
mass in patients with acute myeloid category of spinal tumors is beyond the 1998;19:879-887.
leukemia (AML).28 These are highly scope of this manuscript. 12. Menashe SJ, Iyer RS. Pediatric spinal neopla-
vascular solid masses which, on MRI, sia: A practical imaging overview of intramedullary,
intradural, and osseous tumors. Curr Probl Diagn
appear iso- to hyper-intense on T1- Conclusion Radiol. 2013;42:249-265.
weighted images, and intermediate- to A wide variety of neoplasms can in- 13. Kucia EJ, Maughan PH, Kakarla UK, et al. Sur-
hypo-intense on T2-weighted images, volve the pediatric spine. MRI with gical technique and outcomes in the treatment of
spinal cord ependymomas: part II: myxopapillary
and show marked contrast enhance- contrast is the preferred modality for ependymoma. Neurosurgery. 2011;68 (1 Suppl
ment, as well as diffusion restriction.29 evaluation of spinal cord and spinal canal Operative):90-94.
Similar to focal masses of lymphoma, tumors, while CT scanning is most valu- 14. Moeller KK, Coventry S, Jernigan S, Moriarty
TM. Atypical teratoid/rhabdoid tumor of the spine.
GS can cause effacement of the thecal able for imaging osseous spinal neo- AJNR Am J Neuroradiol. 2007;28:593-595.
sac and cord compression (Figure 18). plasms. Imaging the entire neural axis 15. Vougioukas VI, , Gläsker S, Hubbe U, et al. Sur-
is recommended for spinal cord tumors, gical treatment of hemangioblastomas of the central
nervous system in pediatric patients. Childs Nerv
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