AJR 2000; 174:377-382
© American Roentgen Ray Society
MR Imaging of Spinal Hemangioblastoma
Kim B. Baker1,2,
Christopher J. Moran1,
Franz J. Wippold, II1,3,
James G. Smirniotopoulos3,4,
Fabio J. Rodriguez5,
Steven P. Meyers6 and
Todd L. Siegal7
1
Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington
University School of Medicine, 510 S. Kingshighway Blvd., St. Louis, MO
63110.
2
Present address: Department of Diagnostic Imaging, Temple University, 3401 N.
Broad St., Philadelphia, PA 19104.
3
Present address: Department of Radiology and Nuclear Medicine, Uniformed
Services University of the Health Sciences, 4301 Jones Bridge Rd., Bethesda,
MD 20814.
4
Department of Radiologic Pathology, Armed Forces Institute of Pathology, Bldg.
54, Walter Reed Medical Center, Washington, DC 20306-6000.
5
Department of Radiology, University of Missouri at Columbia, 1 Hospital Dr.,
Columbia, MO 65212.
6
Department of Radiology, Strong Memorial Hospital, University of Rochester
Medical Center, Box 648, Rochester, NY 14642.
7
Department of Radiology, Cooper Hospital, 1 Cooper Plaza, Camden, NJ
08103.
Received January 18, 1999;
accepted after revision June 28, 1999.
Address correspondence to C. J. Moran
Introduction
Hemangioblastoma is a vascular neoplasm of the central nervous system.
Whereas hemangioblastoma is the most common primary neoplasm in the adult
cerebellum, it is a relatively rare tumor of the spine, representing 1.6-5.8%
of all spinal tumors [1].
Hemangioblastoma may occur sporadically or as a component of von Hippel-Lindau
syndrome. Pathologically, hemangioblastoma may be difficult to differentiate
from astrocytoma. Hemangioblastoma may also resemble metastatic renal cell
carcinoma, a lesion that is common in patients with von Hippel-Lindau syndrome
[2]. Radiologic findings are
helpful in diagnosing hemangioblastoma. Preoperative Recognition of
hemangioblastoma may limit the extent of surgery and may alter patient
treatment by indicating von Hippel-Lindau syndrome
[3]. A number of distinct
appearances of spinal hemangioblastoma have been described, including
hemangioblastoma with associated cyst or syrinx
[4], hemangioblastoma with
diffuse spinal cord enlargement
[5], exophytic hemangioblastoma
with minimal spinal cord reaction
[6], and extramedullary
hemangioblastoma [7]. This
pictorial essay presents the characteristic MR imaging findings that may
assist in the preoperative recognition of spinal hemangioblastoma.
Clinical Background
Von Hippel-Lindau syndrome is an autosomal dominant disorder with near
complete penetration and variable expression. The prevalence of von
Hippel-Lindau syndrome ranges from 1:40,000 to 1:50,000
[8]. Von Hippel-Lindau syndrome
manifests as central nervous system hemangioblastomas, renal cysts, and renal
cell carcinomas. Other lesions include retinal angiomas, pheochromocytomas,
pancreatic cysts, and epididymal cystadenomas
[9]. Thirty-two percent of
patients with spinal hemangioblastoma have von Hippel-Lindau syndrome; the
remaining patients have sporadically occurring tumors
[1]. Patients with spinal
hemangioblastoma have a mean age of 30 years at onset, though patients with
von Hippel-Lindau syndrome may present earlier
[1]. Symptoms of spinal
hemangioblastoma are similar to those of other spinal canal tumors and include
sensory change, motor disturbance, and pain
[1]. Because the presentation
of spinal hemangioblastoma is similar to that of other spinal canal masses,
imaging studies can be useful in suggesting a diagnosis.
MR Imaging Characteristics
When compared with the spinal cord, spinal hemangioblastoma is usually
hypointense to isointense on T1-weighted sequences and isointense to
hyperintense on T2-weighted sequences (Fig.
1A,
1B,
1C). T1-weighted images after
gadolinium administration show intense enhancement. Large lesions may be
visualized without contrast material, but small lesions are often isointense
and thus difficult to differentiate from the spinal cord (Figs.
2A,
2B,
2C and
3A,
3B,
3C,
3D). Therefore,
gadolinium-enhanced T1-weighted images are essential for the evaluation of
lesions suggestive of hemangioblastoma.
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Fig. 1A. —45-year-old woman with sporadic cervical hemangioblastoma. 1.5-T
sagittal T1-weighted MR image (TR/TE, 400/11) shows diffusely swollen cervical
spinal cord. Hemangioblastoma posterior to C4 is nearly isointense.
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Fig. 2A. —39-year-old man with von Hippel-Lindau syndrome. 1.5-T T1-weighted
(TR/TE, 550/18) and fast spin-echo T2-weighted sagittal MR images (2400/90)
show abnormal signal intensity and diffuse swelling of spinal cord without
definite tumor identification.
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Fig. 2B. —39-year-old man with von Hippel-Lindau syndrome. 1.5-T T1-weighted
(TR/TE, 550/18) and fast spin-echo T2-weighted sagittal MR images (2400/90)
show abnormal signal intensity and diffuse swelling of spinal cord without
definite tumor identification.
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Fig. 2C. —39-year-old man with von Hippel-Lindau syndrome. Contrast-enhanced
sagittal T1-weighted MR image (550/18) shows three small intensely enhancing
tumors (arrows) in medullary, cervical, and upper thoracic regions.
Patient had prior cervical laminectomy for resection of another
hemangioblastoma.
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Fig. 3A. —35-year-old woman with von Hippel-Lindau syndrome and multiple
spinal hemangioblastomas associated with syrinx. 1.5-T T1-weighted (A)
(TR/TE, 600/15) and fast spin-echo T2-weighted (B) sagittal MR images
(4000/96) show irregular syrinx (asterisks) involving long segment of
thoracic spinal cord. Note flow voids caused by enlarged vessels in
subarachnoid space both anterior and posterior to spinal cord. Small tumors
are not readily apparent without contrast material.
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Fig. 3B. —35-year-old woman with von Hippel-Lindau syndrome and multiple
spinal hemangioblastomas associated with syrinx. 1.5-T T1-weighted (A)
(TR/TE, 600/15) and fast spin-echo T2-weighted (B) sagittal MR images
(4000/96) show irregular syrinx (asterisks) involving long segment of
thoracic spinal cord. Note flow voids caused by enlarged vessels in
subarachnoid space both anterior and posterior to spinal cord. Small tumors
are not readily apparent without contrast material.
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Fig. 3C. —35-year-old woman with von Hippel-Lindau syndrome and multiple
spinal hemangioblastomas associated with syrinx. Contrast-enhanced sagittal
T1-weighted MR image (600/15) shows three intensely enhancing tumors
(arrows).
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Fig. 3D. —35-year-old woman with von Hippel-Lindau syndrome and multiple
spinal hemangioblastomas associated with syrinx. Contrast-enhanced axial
T1-weighted MR image (700/17) through upper lesion shows partially exophytic
tumor (asterisk) of lower thoracic spinal cord with prominent
vascular flow voids (arrows).
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Multiple hemangioblastomas have been described only in patients with von
Hippel-Lindau syndrome [1].
Small hemangioblastomas may be observed in patients with no spinal symptoms or
in relatives of patients with von Hippel-Lindau syndrome. Patients with von
Hippel-Lindau syndrome and spinal symptoms usually have one dominant lesion
that causes symptoms, though other asymptomatic tumors may also be present
(Fig. 4A,
4B,
4C,
4D). Because the presence of
additional hemangioblastomas may alter the therapeutic treatment of the
patient and the patient's family, patients with suspected hemangioblastoma
should undergo contrast-enhanced MR imaging of the entire central nervous
system to exclude multiple lesions
[3].
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Fig. 4A. —41-year-old man with von Hippel-Lindau syndrome, large cervical
hemangioblastoma, and other hemangioblastomas in cervical and thoracic
regions. 1.5-T sagittal T1-weighted MR image (TR/TE, 550/15) shows cervical
and upper portions of thoracic spinal cord to be diffusely enlarged. Large
nodule (asterisk) in posterior aspect of spinal cord at C2 is
partially highlighted by low-signal-intensity syrinx.
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Fig. 4B. —41-year-old man with von Hippel-Lindau syndrome, large cervical
hemangioblastoma, and other hemangioblastomas in cervical and thoracic
regions. Sagittal two-dimensional gradient-echo image (300/18; flip angle,
10°) shows syrinx with higher signal intensity than edematous and swollen
spinal cord. Tumor has mixed signal intensity (arrow).
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Fig. 4C. —41-year-old man with von Hippel-Lindau syndrome, large cervical
hemangioblastoma, and other hemangioblastomas in cervical and thoracic
regions. Sagittal T1-weighted contrast-enhanced MR image (500/15) shows
intensely enhancing nodule at C2. Three small nodules (white arrows)
are observed in cervical region. Large flow voids (black arrows) are
caused by vertebral artery.
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Fig. 4D. —41-year-old man with von Hippel-Lindau syndrome, large cervical
hemangioblastoma, and other hemangioblastomas in cervical and thoracic
regions. Sagittal contrast-enhanced T1-weighted MR image (550/15) of thoracic
region shows three contrast-enhancing tumors (arrows).
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Hemangioblastoma is a highly vascular tumor. MR images of large
hemangioblastomas may show flow voids resulting from prominent vessels (Figs.
3A,
3B,
3C,
3D and
5A,
5B,
5C). Rarely, spinal
hemangioblastoma may hemorrhage in either the cord parenchyma or the
subarachnoid space [10].
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Fig. 5A. —48-year-old woman with sporadic hemangioblastoma and extramedullary
intradural lesion of sacral canal. 0.5-T fast spin-echo T2-weighted sagittal
MR image (TR/TE, 4000/110) of lumbar and sacral spine shows mass behind S1
vertebral body. Note vascular flow voids in spinal canal that envelop lower
thoracic spinal cord and conus medullaris. Tumor signal intensity is similar
to that of cerebrospinal fluid.
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Fig. 5B. —48-year-old woman with sporadic hemangioblastoma and extramedullary
intradural lesion of sacral canal. Contrast-enhanced sagittal T1-weighted MR
image (500/25) shows intensely and homogeneously enhancing tumor. Some
enlarged arteries and veins also enhance.
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Fig. 5C. —48-year-old woman with sporadic hemangioblastoma and extramedullary
intradural lesion of sacral canal. Lateral digital subtraction spinal
arteriography shows large vessel supplying tumor with intense tumor blush.
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The majority of spinal hemangioblastomas originate in the thoracic region,
with cervical lesions also common
[1]. Lumbar and sacral
hemangioblastomas are less frequent (Fig.
5A,
5B,
5C). Hemangioblastomas may
originate from any compartment of the spinal canal or within a vertebral body
[11]. The spinal cord reacts
to hemangioblastoma in a number of different ways; reaction depends on the
size and location of the mass. Sixty-six percent of spinal hemangioblastomas
originate at or near the surface of the spinal cord (either intramedullary
with extension beyond the surface of the spinal cord or exophytic with the
center of the lesion external to the spinal cord). Twenty-five percent of
spinal hemangioblastomas are completely intramedullary and 8% are intradural
extramedullary neoplasms (Baker KB et al., presented at the American Society
of Neuroradiology meeting, June 1997). Fifty-five percent of spinal
hemangioblastomas have an associated cyst or syrinx. Twenty-three percent of
patients have a swollen section of spinal cord distinct from the tumor nodule
and not related to syrinx. Twenty-three percent of patients have no other
spinal cord abnormality (Baker KB et al., ASNR meeting, June 1997).
Descriptions of the different appearances of hemangioblastoma follow.
Exophytic Hemangioblastoma
A hemangioblastoma that originates at the cord surface may incite little or
no reaction in the adjacent spinal cord. The tumor may grow outward in an
exophytic manner (Fig. 6A,
6B,
6C). When the tumor is large,
the appearance may mimic nerve sheath tumors, meningioma, or ependymoma.
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Fig. 6A. —21-year-old woman with sporadic hemangioblastoma and exophytic mass
arising in upper cervical spine and extending to foramen magnum. 1.5-T
contrast-enhanced coronal T1-weighted MR image (TR/TE, 500/28) shows center of
hemangioblastoma is external to spinal cord. Spinal cord is deviated to left
and right and cerebrospinal fluid space (asterisk) is widened.
Low-signal-intensity structure (arrow) is left vertebral artery. Note
absence of associated cyst, syrinx, and spinal cord swelling.
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Fig. 6B. —21-year-old woman with sporadic hemangioblastoma and exophytic mass
arising in upper cervical spine and extending to foramen magnum.
Contrast-enhanced sagittal MR image (500/28) shows extraaxial mass to have
so-called dural tail sign inferiorly (arrowhead) in this off-midline
image. Appearance may be caused by enhancement of epidural veins.
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Fig. 6C. —21-year-old woman with sporadic hemangioblastoma and exophytic mass
arising in upper cervical spine and extending to foramen magnum.
Contrast-enhanced axial T1-weighted MR image (500/28) through C1 level.
Intensely enhancing tumor causes spinal cord (asterisk) to deviate to
left and posteriorly.
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Hemangioblastoma with Syrinx
Hemangioblastoma is known to cause syringomyelia, a condition seen in 40%
of patients [1]. The syrinx may
be small and cystlike or may extend the length of the cord (Figs.
3A,
3B,
3C,
3D,
4A,
4B,
4C,
4D, and
7A,
7B,
7C,
7D). Syrinx is not specific
for hemangioblastoma and is associated with other spinal tumors such as
astrocytoma and ependymoma. Because a small hemangioblastoma can cause a large
syrinx, contrast-enhanced MR imaging is a valuable tool for the diagnosis of
syrinx detected on unenhanced images.
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Fig. 7A. —31-year-old woman with von Hippel-Lindau syndrome and small masses
in thoracic spinal cord with associated cyst. 1.5-T sagittal T2-weighted MR
image (TR/TE, 2200/85) shows intramedullary cavity containing material with
signal intensity parallel to that of cerebrospinal fluid.
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Fig. 7B. —31-year-old woman with von Hippel-Lindau syndrome and small masses
in thoracic spinal cord with associated cyst. Contrast-enhanced T1-weighted
sagittal MR image (700/11) shows two small enhancing nodules (arrows)
in wall of cyst, with central cavity having signal intensity similar to that
of cerebrospinal fluid.
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Fig. 7C. —31-year-old woman with von Hippel-Lindau syndrome and small masses
in thoracic spinal cord with associated cyst. Slightly off-midline
contrast-enhanced T1-weighted sagittal MR image (700/11) shows other small
tumors (arrowheads).
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Fig. 7D. —31-year-old woman with von Hippel-Lindau syndrome and small masses
in thoracic spinal cord with associated cyst. Contrast-enhanced axial
T1-weighted MR image (500/11) shows most inferior enhancing intramedullary
nodule (black asterisk) and portion of cyst (white
asterisk).
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Hemangioblastoma with Associated Spinal Cord Enlargement
A unique characteristic of hemangioblastoma is spinal cord enlargement
beyond the margins of the enhancing tumor and distinct from syrinx
[5] (Figs.
2A,
2B,
2C and
8A,
8B,
8C). Spinal cord swelling may
be caused by arteriovenous shunting, venous congestion, or an edema-promoting
factor produced by the tumor
[5]. When spinal cord
enlargement caused by hemangioblastoma is noted, surgical treatment should be
directed at the enhancing nodule. Spinal cord swelling will usually improve
after the tumor is removed
[5].
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Fig. 8A. —45-year-old woman with sporadic hemangioblastoma and spinal cord
swelling caused by intramedullary tumor. 1.5-T T1-weighted sagittal MR image
(TR/TE, 500/15) shows cervical spine with diffuse swelling and homogeneous
signal intensity.
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Fig. 8B. —45-year-old woman with sporadic hemangioblastoma and spinal cord
swelling caused by intramedullary tumor. T2-weighted sagittal MR image
(2200/80) shows diffuse spinal cord swelling with ill-defined area of high
signal intensity different from cerebrospinal fluid, representing spinal cord
edema. Note C5-C6 disk herniation.
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Fig. 8C. —45-year-old woman with sporadic hemangioblastoma and spinal cord
swelling caused by intramedullary tumor. Contrast-enhanced T1-weighted
sagittal MR image (500/15) shows small enhancing hemangioblastoma
(arrowhead) at C7-T1 level and diffuse spinal cord swelling.
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Extramedullary Hemangioblastoma
Extramedullary hemangioblastoma is rare but may occur in the lumbar or
sacral spinal canal; it may be associated with exiting nerve roots (Figs.
5A,
5B,
5C and
9). In this location the
appearance is similar to that of meningioma or schwannoma. The key to
preoperative diagnosis is the recognition of marked enhancement and associated
enlarged vessels (Fig. 5A,
5B,
5C). A case report described a
hemangioblastoma originating in a vertebral body
[7].
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Fig. 9. —58-year-old man with multiple hemangioblastomas and von
Hippel-Lindau syndrome. Note extramedullary intradural tumor enveloping
thoracic nerve root and filling right neural foramen. 1.5-T contrast-enhanced
T1-weighted axial MR image (TR/TE, 500/20) shows hemangioblastoma in right
neural foramen (arrows). Oblique linear low signal intensity within
tumor probably represents nerve roots.
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Intramedullary Hemangioblastoma with No Spinal Cord Reaction
A small intramedullary hemangioblastoma may not elicit significant cord
enlargement or syrinx (Fig.
7C).
Conclusion
Spinal hemangioblastoma has a number of appearances. Specific appearances
may help differentiate hemangioblastoma from other spinal tumors or vascular
malformations. Hemangioblastoma usually shows uniform contrast enhancement.
Spinal astrocytoma usually shows inhomogeneous enhancement with occasional
central cavitation or hemorrhage
[12]. Spinal ependymoma
usually shows uniform intense enhancement with well-defined margins and
occasional central cavitation
[4]. Spinal astrocytoma or
ependymoma may have associated syrinx, but an extensive syrinx suggests
hemangioblastoma [4]. Neither
spinal astrocytoma nor ependymoma is likely to have prominent vessels
[12]. Spinal cord thickening
remote from the tumor and without syrinx may be specific for hemangioblastoma
[5]. A spinal arteriovenous
fistula may show prominent vessels, cord thickening, and diffuse patchy
enhancement caused by venous engorgement or infarction
[12]; however, spinal
arteriovenous fistulas rarely show a well-defined enhancing mass lesion and
are typically heterogeneous on unenhanced MR images
[4]. Multiple hemangioblastomas
indicate von Hippel-Lindau syndrome
[1].
Acknowledgments
We thank Carolyn Miles for her assistance in the preparation of this
manuscript and Michelle Wynn for her assistance in the preparation of the
photographic images.
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Top
Introduction
Clinical Background
