Eur. Radiol.

9, 1252±1258 (1999) Ó Springer-Verlag 1999

European
Radiology

Review article

Spinal cord tumors

D. L. F. BalØriaux
Clinique de Neuroradiologie, Service de Radiologie, Hôpital Erasme, UniversitØ Libre de Bruxelles, B-1070 Brussels, Belgium

Abstract. Spinal cord tumors are rare; however, every able, occurring in function of tumor localization. Uri-
radiologist should be able to recognize and readily nary disturbance and impotence are rarer and appear
identify those lesions often found in younger patients late in the clinical course of the disease: They occur usu-
or children [1, 2, 3, 4, 5, 6, 7, 8,9]. Early diagnosis plays ally coincident with motor paralysis of the legs.
an important role in the management of the lesions In children pain is equally the most frequent symp-
and interferes with the prognosis and final outcome tom as reported in 42 % of cases. Motor regression is
of the patient [10]. Plain X-ray of the spine and CT present in 36 %, gait abnormality in 27 %, torticollis in
are of limited diagnostic value in cases of intramedul- 27 %, and progressive kyphoscoliosis in 24 % of cases.
lary pathology. Magnetic resonance imaging should Eighty-nine percent of cases are low-grade lesions [10].
be performed as soon as possible and as the first tech-
nique whenever an intrinsic spinal cord lesion is clini-
cally suspected. Systematic analysis of the MR im- Materials and methods
ages together with a basic knowledge of the most
common spinal cord tumors encountered, including Plain films are still routinely performed: abnormal find-
astrocytomas, ependymomas, and hemangioblasto- ings in adults include mainly straightening of the spine
mas, should provide a correct diagnosis and lead to with disappearance of the normal curvatures. Progres-
appropriate treatment subsequently. sive scoliosis should alert the clinician for an underlying
intramedullary lesion that should be ruled out systemat-
Key words: MR imaging ± Spine ± Spinal cord ± Neo- ically. Widening of the spinal canal occurs in children
plasms but not in adults, except in cases of myxopapillary
ependymoma found in young adults and exclusively lo-
cated in the conus medullaris and filum terminale.
Those tumors grow very slowly and may become ex-
Introduction tremely large expanding progressively the spinal canal
and neural foramina.
Tumors of the spinal cord are rare. In a general hospital, If plain films are rarely abnormal in adults with an in-
only 5 % of spinal tumors are intramedullary, whereas tramedullary tumor, they are more often positive in chil-
40 % are intradural extramedullary and 55 % are extra- dren (58±81 %). Computed tomography may show wid-
dural. In children, astrocytomas are by far most fre- ening of the canal as well when present. Plain CT should
quent, whereas in adults, ependymomas represent 60 % not be chosen as a screening modality for intraspinal le-
of the intramedullary tumors. Astrocytomas account sions as visualization of the canalar content is still limit-
for approximately 30 % of spinal cord glioma. Hem- ed.
angioblastomas are less frequent and represent 5 %. Myelography has been the preferred imaging modal-
Clinical presentation is poorly specific with pain be- ity for the visualization of the spinal cord. This method
ing the most common finding and usually the first symp- should definitely be avoided because the patient's symp-
tom to be reported. Sensory and motor deficit are vari- tomatology may occasionally be exacerbated by the
procedure. Moreover, only the cord contours are delin-
Present address: D. L. F. BalØriaux, 808 Route de Lennik,
eated with myelography and no information is obtained
B-1070 Brussels, Belgium concerning the intramedullary appearance.
Presently, MRI is definitely the imaging procedure of
Categorical Course ECR 2000 choice for the optimal demonstration of the cord. The
D. L. F. BalØriaux: Spinal cord tumors 1253

a b

Fig. 1a±d. Cervical cord ependymoma. a Sag-

ittal T1-weighted, b T2-weighted, c post-con-
trast T1-weighted, and d axial post-contrast
T1-weighted images. The cervical spine is
straightened. The spinal cord is enlarged
from C2 to C7. The solid component is spon-
taneously slightly hyperintense on the T1-
weighted image, inhomogeneously iso- to
hyperintense on T2-weighted images, and
enhances strongly after intravenous contrast
injection. At both ends, associated cysts are
visible: Their contours do not enhance and
the signal behavior is similar to that of cere-
brospinal fluid (CSF). Associated edema is
seen only at the level of C1±C2 as an ill-de-
fined mildly hyperintense area (arrow). The
c d axial view shows the centromedullary loca-
tion of the tumor best

MRI technique should be performed in any patient in tensive in hemangioblastomas. It is seen in 60 % of all
whom an intramedullary pathology is clinically suspect- ependymomas and only in 23 % of astrocytomas. Intra-
ed. tumoral cystic components are located entirely within
The MR technique must include systematically T1- the boundaries of the tumor with contrast enhance-
and T2-weighted images as well as gadolinium-en- ment frequently observed in the cyst wall. The signal
hanced T1-weighted images [11]. The entire spinal cord behavior of these cysts is usually different from cere-
must be studied with dedicated phased-array spinal sur- brospinal fluid (CSF), due to the high protein content
face coils. At least two different imaging planes must of the fluid. On the contrary, associated, ªsatelliteº
be used in order to locate the tumor properly and to dif- cysts are located cephalad or caudal to the solid nodule
ferentiate intra- from extramedullary tumors. and their walls do not enhance after gadolinium injec-
Systematic analysis of the MR images must be done tion. They develop as an intramedullary reaction to
and both, morphological anomalies as well as signal-be- the presence of the tumor and the liquid has a behavior
havior anomalies, must be described in order to identi- similar to that of CSF. They spontaneously collapse af-
fy the extent of the tumor and separate tumor compo- ter surgery.
nents (Fig. 1). It is of the utmost importance to differ- Finally, extensive associated hydromyelia may devel-
entiate tumor infiltration from associated edema, and op as well: those cavities will equally disappear sponta-
solid nodule from cystic elements. Edema is most ex- neously after total tumor removal.
1254 D. L. F. BalØriaux: Spinal cord tumors

Solid tumor components have either clear limits, as to chronic hemorrhage (Fig. 3). Ependymomas do en-
in the case of low-grade tumors such as ependymomas or hance vividly and homogeneously, and have well-de-
hemangioblastomas, or ill-defined borders as in the case fined borders [1, 2, 4, 12, 13].
of more infiltrating and more aggressive tumors. Ependymomas originate from the ependymal walls
Contrast enhancement patterns should also be care- and as such are more centromedullary located com-
fully described: ependymomas usually enhance more in- pared with astrocytomas (Fig. 1). The mean size of the
tensely and homogeneously, whereas astrocytomas en- ependymoma corresponds to a mean height of three to
hance moderately and in a inhomogeneous way. four vertebral bodies (min. 2, max. 13), whereas astrocy-
It is important to know that not all tumors do en- tomas are usually more extensive, with a mean height of
hance, whereas on the contrary, any enhancing intra- 5.6 vertebral bodies (min. 2, max. 19).
medullary mass expanding the cord must not be consid-
ered as being a neoplasm.
Bearing in mind the need for this systematic image Hemangioblastoma
analysis the radiologist can provide useful preoperative
information to the neurosurgeon: the histology may be Hemangioblastomas are richly vascularized tumors,
suspected, the tumor borders assessed, and surgical usually located eccentrically. These tumors can be either
planning facilitated. solitary or multiple (if associated with von Hippel-Lin-
Hemorrhage may occur within spinal cord tumors dau disease). Tumor nodules are usually small and they
and can be recognized on T1-weighted images as hyper- are typically associated with extensive hydrosyringomy-
intense areas, when the hemorrhage is 1 week to approx- elia. When no cystic component is present, extensive
imately 4 months old. Hemosiderin deposits can later be edema is usually found. For these reasons hemangio-
identified as low-signal areas on T2-weighted images, blastomas are often easily identified [14, 15].
preferably obtained by gradient-echo sequences.

Less frequent tumors

Most common spinal cord tumors
Lymphoma
Astrocytomas, ependymomas, and hemangioblastomas
are by far the most frequent intramedullary tumors [1, An intramedullary lymphoma may occur as part of a
4,12]. Astrocytomas are more often located in the tho- multifocal lymphoma, with cerebral, cerebellar, or brain
racic region. Moreover, a panmedullary infiltrating tu- stem lesions associated with the intramedullary lesion.
mor is usually an astrocytoma. Ependymomas are more Primary intramedullary lymphoma is rare.
frequently found in the cervical region. Hemangioblas-
tomas can be found at any level.
Metastasis

Astrocytoma Four percent of intramedullary tumors are metastases.

Still, it is difficult to ascertain the true incidence of spi-
Astrocytomas are common tumors and account for 24 to nal cord metastases as the clinical picture often is atypi-
30 % of intramedullary tumors in adults, whereas it is by cal and seen in terminally ill patients [16].
far the most common tumor in children (up to 90 %) Autopsy material is also biased as the cord is often
Even in adults, they are mostly encountered in younger not systematically examined. On the other hand, 2.4 %
patients (mean age in our series was 29 years) with a of metastases removed surgically from the central ner-
predominance of males (63 %). vous system are located in the cord. Clinical symptoms
The typical astrocytoma is large and located in the are often nonspecific but usually involve root pain.
thoracic region. If a cystic component is present, it is The extreme sensitivity of MRI enables intramedul-
typically an intratumoral one; however, satellite cysts lary metastases to be detected easily. No specific MRI
and secondary hydromyelia are also observed. characteristics are seen. Usually they are small, nodular,
The astrocytoma is usually eccentrically located and well-defined lesions, hyperintense on T2-weighted im-
exhibits heterogeneous, moderate, and partial contrast ages. The enhancement pattern may be either ring-like
enhancement after gadolinium injection. Astrocytoma or homogeneous and intense (Fig. 4).
borders are frequently ill-defined (Fig. 2).

Ganglioglioma
Ependymoma
Gangliogliomas are rare tumors, representing 3.8 % of
The typical ependymoma is located in the cervical re- all central nervous system involving the upper cervical
gion and associated large satellite cysts and frequently cord in the great majority of cases [17, 18]. One third of
seen. A so-called cap sign is often seen and corresponds the gangliogliomas are seen in children of which spinal
to low-signal-intensity areas capping at both sides the cord involvement (1.7 %) is greater than cerebral
tumor limits: these caps are hemosiderin deposits due (1.4 %) or cerebellar (0.7 %) involvement.
D. L. F. BalØriaux: Spinal cord tumors 1255

2a 2b 2c

Lipoma

Lipomas are relatively rare spinal cord tumors, repre-

senting 6 % of intramedullary tumors in our series.
True intramedullary lipoma must be differentiated
from cauda equina lipomas or lipomas associated with
dysraphism. The clinical, radiological, and surgical
problems raised by these lesions are totally different.
Although these tumors appear well-defined on MRI,
often no cleavage plane from the surrounding spinal
cord is observed at surgery; therefore, the tumor usually
cannot be completely resected without causing severe
neurological damage.
The typical hyperintensity of lipomas on T1-weight-
ed images makes these lesions easy to diagnose with
MRI [19].
3
Fig. 2a±c. Cervical low-grade astrocytoma. a Sagittal T1-weighted, Cavernoma
b T2-weighted, and c post-contrast T1-weighted images. The cervi-
cal spinal cord is enlarged by a C1±C6 tumor, hyperintense on T2- Cavernomas are vascular malformations that may re-
weighted images, and faintly seen on T1-weighted images as an
ill-defined low-signal-intensity area. No contrast uptake is seen af-
main clinically silent for a long time. Often they are re-
ter intravenous gadolinium injection sponsible for an acute or rapidly progressive neurologi-
cal medullary deficit. Cavernomas represent 2.4 % of
Fig. 3. Cervical ependymoma: the ªcapº sign. This sagittal T2- all intramedullary tumors. All cases presented with
weighted image shows the cervical tumor ªcappedº at both sides hemorrhage. Before the advent of MRI, these lesions
by a hypointense rim (arrow) corresponding to hemosiderin depos- were extremely difficult to diagnose, especially in the
its due to chronic hemorrhage spinal cord, as they usually are small and do not enlarge
the spinal cord [1, 4, 20].
On the other hand, on MRI, intramedullary caverno-
Oligodendroglioma mas (or cavernous hemangiomas) are usually easily rec-
ognized. A reticulated appearance with areas of mixed
Oligodendrogliomas are rare in the spinal cord. They signal intensity in both T1- and T2- or T2*-weighted im-
exhibit no specific MRI characteristics; however, the ages is the most common finding. A prominent rim of
few cases in our experience were relatively small tu- decreased signal intensity is less commonly seen than in
mors (two vertebral segments), with ill-defined bor- the brain. Contrast enhancement may occur (Fig. 5). As
ders and slight hyperintensity on T1-weighted images. cavernomas may be multiple, we recommend cerebral
No peritumoral edema or contrast enhancement was MRI whenever the diagnosis of cavernoma is suspected.
seen. If multiple similar lesions are found in the brain, this
1256 D. L. F. BalØriaux: Spinal cord tumors

a b c d
Fig. 4a±d. Multiple intramedullary metastases from a lung carcino- on T2-weighted images and enhance strongly after gadolinium in-
ma. a,b Adjacent sagittal T2-weighted and c,d post-contrast T1- jection. The spinal cord shows multiple focal sites of enlargement
weighted images. The multiple metastatic tumors are hypersignal producing a ªmoniliformº aspect

a b c d
Fig. 5a±d. Spinal cord cavernoma. a Sagittal T1-weighted, b T2- a heterogeneous signal on all pulse sequences. Only mild contrast
weighted, c post-contrast T1-weighted, and d coronal post-contrast enhancement is seen. On T2-weighted images, note the presence
T1-weighted images. The cavernoma is responsible for a focal ma- of a cap sign at the upper limit of the lesion (arrow)
jor enlargement of the cord at the level of C2±C3. The lesion has

should support the final diagnosis of cavernoma of the senting as an intramedullary tumor. Magnetic resonance
spinal cord. imaging demonstrated nonspecific findings of a nodular
Finally, it should be stressed that numerous nonneo- lesion which usually strongly enhances on gadolinium-
plastic intramedullary lesions may simulate tumor infil- enhanced T1-weighted images (Fig. 6). Sarcoidosis
tration and be distinguished properly in order to avoid should thus be included in the differential diagnosis of
unnecessary biopsy. Typically, those lesions include MS a nodular lesion. Biopsy may be required and should
plaques, inflammatory lesions, granulomas, and ab- be performed to establish the diagnosis when no system-
scesses. Sarcoidosis is rare in the spinal cord [21]. Diag- ic signs of sarcoidosis are present.
nosis of an intramedullary lesion is facilitated when the
patient has known systemic sarcoidosis. In two of our
cases the patient had a solitary spinal cord lesion pre-
D. L. F. BalØriaux: Spinal cord tumors 1257

a b

Fig. 6a±d. Cervical spinal cord sarcoidosis. a Sagittal T1-weighted,

b T2-weighted, c post-contrast T1-weighted, and d axial post-con-
trast T1-weighted images. There is a mild cord enlargement at the
level of C2±C4. The lesion demonstrates high signal intensity on
T2-weighted images, is isosignal to the cord on T1-weighted im-
ages, and shows definite enhancement after contrast injection.
c The axial images are useful to better show the left lateral location
of the lesion (arrow)
1258 D. L. F. BalØriaux: Spinal cord tumors

Conclusion 6. Przybylski GJ, Albright AL, Martinez AJ (1997) Spinal cord

astrocytomas long-term results comparing treatments in chil-
dren. Child Nerv Syst 13: 375±382
It is usually not possible to provide a diagnosis of spinal
7. Russo CP, Katz DS, Corona RJ Jr, Winfield JA (1995) Ganglio-
cord tumor with absolute certainty, and biopsy should cytoma of the cervicothoracic spinal cord. Am J Neuroradiol
always be performed to establish the final histological 16: 889±891
diagnosis; still, the radiologist will suggest a histological 8. Sibilla L, Martelli A, Farina L, Uggetti C, Zappoli F, Sessa F,
diagnosis and discuss the differential diagnosis. Rodriguez y Baena R, Gaeltani P (1995) Ganglioneuroblasto-
The radiologist is unable to predict the resectability ma of the spinal cord. Am J Neuroradiol 16: 875±877
9. Sze G (1996) Neoplastic disease of the spine and spinal cord.
of a tumor. In some cases, although no clear separation
In: Atlas SW Magnetic resonance imaging of the brain and
between tumor and normal spinal cord is observed on spine, 2nd edn. Lippincott-Raven, Philadelphia, 1339±1385
MRI, the surgeon will discover a cleavage plane at sur- 10. Constantini S, Houten J, Miller DC, Freed D, Ozek MM,
gery. Alternatively, a clearly definable tumor on MRI Rorke LB, Allen JC, Epstein FJ (1996) Intramedullary spinal
may not be easily resectable, as in the case of spinal lipo- cord tumors in children under the age of 3 years. J Neurosurg
ma, where MRI shows sharp tumor margins, whereas 85: 1036±1043
11. Bydder GM, Brown J, Niendorf HP, Young IR (1985) Enhance-
the surgeon finds diffuse infiltration with no separation
ment of cervical intraspinal tumors in MR imaging with intra-
between cord and fatty tissue [2]. venous gadolinium-DTPA. J Comput Assist Tomogr 9: 847±851
In case of diagnostic uncertainty, it may be useful to 12. Fine MJ, Kricheff II, Freed D, Espstein FJ (1995) Spinal cord
perform a baseline study and then follow-up MR exam- ependymomas MR imaging features. Radiology 197: 655±658
inations of the spinal cord rather than a biopsy. Spinal 13. Kahan H, Sklar EML, Donovan Post MJ, Bruce JH (1996) MR
cord tumors are usually slow-growing lesions and have characteristics of histopathologic subtypes of spinal ependymo-
ma. Am J Neuroradiol 17: 143±150
a relatively stable appearance on MRI.
14. Colombo N, Kucharczyk W, Brant-Zawadzki M, Norman D,
Scotti G, Newton TH (1986) Magnetic resonance imaging of
spinal cord hemangioblastoma. Acta Radiol (Diagn)
References 769:S734±S737
15. Minami M, Hanakita J, Suwa H, Suzui H, Fujita K, Nakamura
1. BalØriaux D, Parizel P, Bank WO (1992) Intraspinal and intra- T (1998) Cervical hemangioblastoma with a past history of sub-
medullary pathology. In: Imaging of the spine and spinal cord. arachnoid hemorrhage. Surg Neurol 49: 278±281
Manelfe C (ed) Raven Press, New York, pp 513±564 16. Ijaz T, Jones K (1997) Images in clinical medicine. Intramedul-
2. BalØriaux D MRI of spinal cord diseases. CD-ROM, Lasion, lary spinal cord metastases. N Engl J Med 13: 336±768
2nd edition 17. Hamburger C, Buttner A, Weis S (1997) Ganglioglioma of the
3. Bourgouin PM, Lesage J, Fontaine S, Konan A, Roy D, Bard spinal cord report of two rare cases and review of the literature.
C, Del Carpio O'Donovan R (1998) A pattern approach to the Neurosurgery 41: 1410±1415
differential diagnosis of intramedullary spinal cord lesions on 18. Patel U, Pinto RS, Miller DC, Handler MS, Rorke LB, Epstein
MR imaging. Am J Roentgenol 170: 1645±1649 FJ, Kricheff II (1998) MR of spinal cord ganglioglioma. Am J
4. Brotchi J, Dewitte O, Levivier M, BalØriaux D, Vandesteene Neuroradiol 19: 879±887
A, Raftopoulos C, Flament Durand J, Noterman J (1991) A 19. Timmer FA, van Rooij WJ, Beute GN, Teepen JL (1993) Intra-
survey of 65 tumors within the spinal cord: surgical results and medullary lipoma. Neuroradiology 38: 159±160
the importance of preoperative magnetic resonance imaging. 20. Lahanis S, Vlahos L, Gouliamos A, Papavasiliou C (1993) Ar-
Neurosurgery 29: 651±657 teriovenous malformation of the spinal cord mimicking a tu-
5. Papadatos D, Albrecht S, Mohr G, del Carpio-O'Donovan R mour. Neuroradiology 35: 598±599
(1998) Exophytic primitive neuroectodermal tumor of the spi- 21. Levivier M, Brotchi J, BalØriaux D, Pirotte B, Flament-Durand
nal cord. Am J Neuroradiol 19: 787±789 J (1991) Sarcoidosis presenting as an isolated intramedullary
tumor. Neurosurgery 25: 271±276