Case record...Vertebral hemangioma with multisegmental retromedullary lipoma

1. CASE OF THE WEEK
PROFESSOR YASSER METWALLY
CLINICAL PICTURE
CLINICAL PICTURE:
A 51 years old female patient presented clinically with paraplegia with a dorsal sensory level of gradual onset and
progressive course.
RADIOLOGICAL FINDINGS
RADIOLOGICAL FINDINGS:
Figure 1. Dorsal vertebral hemangioma with epidural extension and fat overgrowth. Postcontrast CT scan of D4
vertebra showing the characteristic appearance of vertebral hemangioma of alternating coarse trabeculae and low-
density cystic areas. Notice the dotted appearance of the vertebral, mostly due to destruction of transverse trabeculae
and preservation and thickening of the longitudinal trabeculae. Such haemangiomas are usually of the cavernous type.
There is also resorption of bone, with replacement by sinusoids, and thickening of remaining trabeculae. The cortical
margin is intact in most cases and may bulge at the posterior aspect. Histologically, many abnormal vascular channels
of varying calibre are seen interspersed within a fatty matrix and thickening of vertical trabiculations. The coarse,
vertical, and thickened trabecular pattern, with osseous reinforcement (trabecular thickening) adjacent to the
vascular channels cause bone resorption and reactive fat overgrowth in between the thickened trabeculae. Reactive fat
overgrowth in between the thickened trabeculae is responsible for the radiolucency that is observed between the
hyperdense thickened trabeculae. This appearance (the dot appearance.... trabecular thickening) on radiographs
represents a response to stress and has been likened to corduroy. Vertebral fractures at the site of these hemangiomas
are unusual because of this trabecular reinforcement and thickening.

2. Figure 2. Post intravenous contrast CT scan image (A) and CT myelography (B,C). Notice the epidural extension of
the hemangioma that showed contrast enhancement (A). The remnant of dye in the subarachnoid spaces (B,C) showed
that the spinal cord is actually pushed anteriorly by AN epidural mass of fat density (epidural lipoma)
Figure 3. Sagittal reconstruction images showing the vertebral bony hemangioma with enhanced epidural extension
and the multisegmental retromedullary lipoma pushing the spinal cord anteriorly. The retromedullary lipoma is
probably reactive to the epidural hemangioma rather than a true neoplasm. Operative finding, however, revealed an
epidural angiolipoma.

3. Figure 4. CT myelography showing the vertebral hemangioma and the multisegmental retromedullary lipoma pushing
the spinal cord anteriorly. The retromedullary lipoma is probably reactive to the epidural hemangioma rather than a
true neoplasm. Operative finding, however, revealed an epidural angiolipoma.
Figure 5. A, Plain CT scan, B, CT scan with intravenous contrast showing a vertebral bony hemangioma extending
into the epidural spaces. quot;vertebral hemangiomas are rarely implicated in symptoms formation (unless proved
otherwise by radiological studies)quot;. Notice the epidural retromedullary lipoma pushing the spinal cord anteriorly and
apparently is the cause of compressive myelopathy in this case. The retromedullary lipoma is probably reactive to the
epidural hemangioma rather than a true neoplasm. Operative finding, however, revealed an epidural angiolipoma.

4. Figure 6. Plain x ray showing a vertebral body haemangioma. The vertebral bodies appear dotted in cross-section due
to destruction of transverse striation and preservation of the longitudinal striation. These lesions are well
demonstrated by plain CT scan and do not enhance after i.v. contrast injection. The vertebral hemangioma in this
patient was not causely related to the paraplegic state. The cause of paraplegia was an epidural retromedullary lipoma
pushing the spinal cord anteriorly, which when debulked surgically the patient much improved. On plain radiographs
and CT, there is exaggeration of the vertebral trabeculae of the vertebral body (also called quot;accordionquot; or
quot;honeycombquot; vertebrae). This appearance may still be preserved after vertebral collapse. The cortical margin is
intact in most cases and may bulge at the posterior aspect
Figure 7. A, Plain CT scan, B, CT myelography showing a vertebral body
haemangioma associated with an extradural, retromedullary lipoma pushing
the spinal cord anteriorly. On plain radiographs and CT, there is exaggeration
of the vertebral trabeculae of the vertebral body (also called quot;accordionquot; or
quot;honeycombquot; vertebrae). This appearance may still be preserved after
vertebral collapse. The cortical margin is intact in most cases and may bulge
at the posterior aspect
Figure 8. Operative findings revealed an epidural angiolipoma, following debulking of this lipoma the patient much
improved.

5. Box 1. Characteristics of vertebral hemangiomas
 Histologically, many abnormal vascular channels of varying calibre are seen interspersed within a fatty matrix
and thickening of vertical trabiculations.
 The coarse, vertical, trabecular pattern, with osseous reinforcement (trabecular thickening) adjacent to the
vascular channels that have caused bone resorption. This appearance on radiographs represents a response to
stress and has been likened to corduroy. Vertebral fractures at the site of these hemangiomas are unusual
because of this trabecular reinforcement.
 Fatty overgrowth are probably reactive in nature secondary to the hemangioma and probably it
represents a response to stress.
 The vascular channels frequently causes bone resorption that progress to destruction of horizontal
trabeculae.
 Thickening of the vertical trabeculae, with osseous reinforcement represents a response to stress.
Trabecular thickening causes vertebral reinforcement thus making vertebral fractures at the site of these
hemangiomas unusual.
 The dotted appearance of CT scan imaging is due to the thickened vertical trabeculae.
 Trabeculae thickening occurs through intramembranous bone formation adjacent to the angiomatous
channel.
 The radiolucency interspersed between the hyperdense dots is due to increased fat content in between the
thickened vertical trabeculae. Bone resorption and cystic changes probably play a part in this
radiolucency but to much lesser extent, and that is why vertebral hemangiomas appear hyperintense on
non-contrast MRI T1 images due to increased fat content.
 The multisegmental epidural lipoma observed in this case is probably reactive to the epidural extension
of the hemangioma rather than true neoplasm. Lipomas are neither true tumours nor they are
hamartomas, they are simply normal adipose tissues in abnormal sites. The epidural lipoma, whatever its
aetiology and pathogenesis, has apparently reached a large size enough to exert significant spinal cord
compression and myelopathy.
 Although epidural, extraosseous extension of the vertebral hemangioma is observed in this case, however
it was not causing any spinal cord compression or displacement as CT myelography demonstrated that
the spinal cord is pushed anteriorly by the retromedullary lipoma rather than posteriorly by the epidural
hemangioma. One can not, however, negates the possible role of a vertebral hemangioma in symptom
formation by causing bleeding with epidural hematoma or vascular steal with spinal cord ischemia.
DIAGNOSIS:
DIAGNOSIS: VERTEBRAL HEMANGIOMA WITH MULTISEGMENTAL EXTRADURAL
RETROMEDULLARY LIPOMA
DISCUSSION
DISCUSSION:
 Vertebral hemangioma
Haemangioma of the vertebra is a benign, slow growing tumour of the blood vessels. It makes up 30% of all
haemangiomas of bone and is found in approximately 10% of autopsies. It is commonly seen in women over 40 and
has a predilection for the lower thoracic or upper lumbar spine. Most vertebral haemangiomas are asymptomatic and
are of no clinical significance. However vertebral collapse or extension into the spinal canal may result in pain, spinal
cord compression and/or paraplegia. Histologically, many abnormal vascular channels of varying calibre are seen
interspersed within a fatty matrix. The haemangioma is usually of the cavernous type. There is also resorption of
bone, with replacement by sinusoids, and thickening of remaining trabeculae.

6. Hemangioma of bone histologically resembles cavernous hemangiomas (cavernomas). Radiographically it has a
pathognomonic appearance of alternating coarse trabeculae and low-density cystic areas. The alternating pattern is
particularly well seen on CT. Hemangiomas typically arise in the vertebral body, unlike osteoid osteoma,
osteoblastoma, and aneurysmal bone cyst, which arise in the posterior neural arch. Although most hemangiomas of
the spine are discovered as incidental findings, they have been reported to cause vertebral canal compromise. They
can grow over time and have been associated with hematoma in cases of trauma. On MRI the lesion is hyperintense on
the precontrast MRI T1 studies, and On MRI T2 studies the lesion appears of heterogenous signal intensity, probably
due to the presence of blood products. The author did not observe enhancement of these lesions following contrast
injection.
Figure 9. Asymptomatic spinal hemangioma at T-6 in a 67-year-old man. A
Lateral conventional tomogram shows typical trabecular thickening (arrows) at
the site of the hemangioma. (B and C) Photographs of the macerated dry bone (B)
and gross specimens sectioned coronally (C) demonstrate the trabecular
thickening (arrowheads). (D) Photomicrograph (original magnification,
approximately X5; hematoxylin-eosin stain) also shows trabecular thickening
(solid arrows), angiomatous components (open arrows), and fat overgrowth
(arrowheads), compared with the normal, more cellular marrow (*) of adjacent
vertebrae. Trabeculae thickening occurs through intramembranous bone
formation adjacent to the angiomatous channel.
These tumours are commonly found in the vertebral bodies and laminae, cavernous hemangiomas are slowly growing,
small benign lesions that have been demonstrated in 11% of spines at autopsy, but are only rarely symptomatic. They
occur in the body of the vertebra, although the posterior elements can be affected. Rarely, compression of neural

7. elements by dilated epidural and paraspinal draining veins can occur in association with hypervascular hemangiomas.
They occur most commonly in the thoracic spine. Radiologically, these are very distinct lesions of the bony casing of
the spinal cord that, in time, will expand into the epidural space and produce a compressive myelopathy.
In the author experience, vertebral hemangiomas are rarely implicated in symptoms formation (unless proved
otherwise by radiological studies) and even when discovered in a paraplegic patient a search for anther cause of
paraplegia must be made. See figs. 12,13
On plain radiographs and CT, there is exaggeration of the vertebral trabeculae of the vertebral body (also called
quot;accordionquot; or quot;honeycombquot; vertebrae). This appearance may still be preserved after vertebral collapse. The cortical
margin is intact in most cases and may bulge at the posterior aspect. The vertebral haemangioma may have
extraosseous extension and surround the cord several levels above the bone lesion. Cord compression usually occurs in
middle aged adults, sparing children and the older age group. Paravertebral soft tissue extensions are occasionally
seen. The vertebral bodies appear dotted in cross-section due to destruction of transverse striation and preservation of
the longitudinal striation. These lesions are well demonstrated by plain CT scan and do not enhance after i.v. contrast
injection.
A characteristic appearance is usually seen on MRI. A mottled pattern with increased signal intensity is seen on T1
weighted images due to fatty tissue interspersed with the thickened trabeculae. Extraosseous tumour, if present, is
better evaluated with MRI than with any other imaging modality.
Appearance of exaggerated vertical trabeculae as described above may also be seen in secondary osteoporosis,
multiple myeloma, lymphoma, metastases, Paget's disease or blood dyscrasia. Hence, any symptomatic lesion of the
vertebrae with features of a haemangioma must be carefully evaluated.
Figure 10. Plain x ray showing a
vertebral body haemangioma, B, A
characteristic appearance is usually
seen on MRI. A mottled pattern with
increased signal intensity is seen on T1
weighted images due to fatty tissue
interspersed with the thickened
trabeculae.
Vertebral hemangiomas are the most common primary spinal neoplasm and are present in 10 to 12% of the
population, based on autopsy studies performed in adults.[4,7,29,31,37] Usually incidental, asymptomatic, and
solitary, these are benign vascular lesions that can give rise to symptoms in rare circumstances. Symptoms include
radicular pain in most cases, and neurological compromise can occur in up to 40% of symptomatic cases. Symptoms
are thought to develop by the following mechanisms: 1) vascular expansion of a vertebral body, pedicle, lamina, or
facet leading to direct compression of nerve roots and/or the spinal canal; 2) subperiosteal extension of the
hemangioma resulting in an extradural mass compressing the spinal cord; and 3) compression fractures and
vertebral collapse secondary to replacement of bone with hemangioma. Rarely, a vertebral hemangioma may cause
bleeding with epidural hematoma or vascular steal with spinal cord ischemia.
Vertebral hemangiomas exhibit a classic radiographic appearance of coarse vertical striations, referred to as a

8. honeycomb pattern, on plain spine x-ray films or CT scans. Conventional MR imaging is less definitive for
hemangioma, but the high blood content of the lesion demonstrates high T2-weighted signal intensity on fat-
suppression sequences. Angiograms can also aid in diagnosis and provide a road map for treatment (that is,
embolization). The vascular supply to vertebral hemangiomas usually originates from small branches of the
intercostal or lumbar arteries that arise proximal to the radicular branches. Certain radiographic features such as
neural arch involvement, complete vertebral body involvement, expanded osseous cortex with indistinct margins, an
irregular honeycomb pattern of bone, an epidural mass, and fatty stroma are associated with the development of
symptoms,[22] which most pregnancy-related cases demonstrate.
The histological appearance of hemangiomas consists of benign vascular proliferation with normal capillary and
venous structure.[8,29,35] Two types have been described: cavernous or capillary. The most common type of
vertebral hemangioma is the cavernous type, which is characterized by large sinusoidal spaces lined by a single layer
of epithelium. The capillary type of vertebral hemangioma differs from the cavernous type only by having smaller
vascular channels. Whereas asymptomatic hemangiomas occur primarily in the thoracolumbar spine, symptomatic
hemangiomas most commonly occur at the thoracic level.[27] Lumbar or cervical locations for symptomatic
hemangiomas are exceedingly rare.
 Epidural lipoma
These tumours are situated in the dorsal region in the extradural retromedullary spaces. They usually extend for more
than one vertebral segment and are well demonstrated by plain CT scan as regions of fat density. No significant
enhancement occurs after i.v. contrast injection. Lipomas are occasionally associated with bony haemangiomas and
are considered reactive to it. The larger the degree of fat overgrowth in the stroma between thickened trabeculae (seen
on CT images as low attenuation between thickened trabeculae and as areas of high intensity on T1-weighted images
and intermediate intensity on T2-weighted images), the less likely these lesions will be symptomatic (inactive
hemangioma) as shown by Laredo and coworkers [50].
Lipomas originate from the focal premature dysjunction of neural ectoderm from cutaneous ectoderm that allows
migration of periaxial mesoderm into the developing neural tube. This pluripotent mesoderm primarily develops into
fat, but may also develop into other tissues, including blood vessels. So the combined lesion might be explained by the
focal maturation of this pluripotent mesoderm, which migrates between the two ectodermal layers and primarily
matures as a lipoma in the primitive vascular plexus. Subsequent failure to form the capillary component in this
plexus might lead to the formation of a combined lesion (vascular lesion combined with epidural lipoma) [39,40].
Overgrowth of adipose tissue is most frequently associated with hemangiomas and are considered reactive to it, and
this characteristic led some authors in the past to refer to these lesions as angiolipomas. However, fat overgrowth
should be considered a reactive phenomenon as opposed to a true neoplastic component; therefore, the term
angiolipoma is not appropriate for the vast majority of these musculoskeletal vascular lesions [41,42,43]. True
angiolipomas are rare lesions.
Operative debulking of excessive epidural fat (whither it is considered reactive to the hemangioma or a true neoplasm)
is probably indicated and might be useful to the patient especially when it exerts significant spinal cord displacement
and compression.
Box 2. Radiographic features that is associated with development of symptoms in vertebral hemangiomas
1. Neural arch involvement
2. Complete vertebral body involvement
3. Expanded osseous cortex with indistinct margins
4. An irregular honeycomb pattern of bone
5. An epidural mass, and fatty stroma

9.
Figure 11. Hemangioma of L3 in a 65-year-old man.
Nearly the entire body is occupied by this predominantly
lytic lesion, which has multiple areas of distinct high
density representing thickened trabeculae. The
demonstrated radiolucency is due to increased fat
content in between the thickened trabeculae.
Figure 12. Epidural lipoma associated with multiple spinal haemangiomas. The vertebral hemangiomas are an
incidental discovery in this patient and was not causely related to the paraplegic state of the patient. Spinal cord
compression by the epidural lipoma was the cause of paraplegia in this patient.

10. Figure 13. A MRI precontrast T1 (A) AND MRI T2 (B,C,D,E) studies showing a case of low dorsal cord compression.
MRI showed multiple vertebral hemangiomas (the lesions are hyperintense on the precontrast MRI T1 image [A] and
of heterogenous signal intensity on the MRI T2 images). The vertebral hemangiomas in this patient were not causely
related to the paraplegic state. The cause of paraplegia was a heavily calcified dorsal disc herniation compressing the
spinal cord (C,D,E), which when removed surgically the patient much improved.

11. Figure 14. Asymptomatic hemangioma at L-3 in a 30-year-old man. A Axial T1-weighted MR image shows low-signal-
intensity thickened trabeculae (arrowheads) in a background of fat overgrowth (arrows). B Sagittal conventional spin-
echo T2-weighted MR image reveals high signal intensity in the vascular portions of the lesion (arrows), although the
majority of the lesion appears similar to adipose tissue because of the large amount of fat overgrowth.
SUMMARY
SUMMARY
 Osseous Hemangioma
The majority of hemangiomas that involve bone are discovered incidentally in asymptomatic patients. Men are
affected twice as often as women, and lesions are usually discovered in the 4th 5th decades of life. Soft-tissue
components may also be associated with these lesions. Osseous hemangioma is particularly common in the spine and
calvaria and less frequently affects long bones such as the tibia, femur, and humerus.
Vertebral hemangioma is extraordinarily common, seen in 11% of the cases in one large autopsy series [45]. It
accounts for 28% of all skeletal hemangiomas [46]. These lesions can involve only a portion of or the entire vertebral
body and are multiple in one-third of the cases [47]. The thoracic spine is the most common location for vertebral
hemangiomas. [44,45,46,47].
At radiography, vertebral hemangiomas classically have a coarse, vertical, trabecular pattern, with osseous
reinforcement (trabecular thickening) adjacent to the vascular channels that have caused bone resorption [48]. This
appearance on radiographs represents a response to stress and has been likened to corduroy. Vertebral fractures at
the site of these hemangiomas are unusual because of this trabecular reinforcement. At CT, the thickened trabeculae
are seen in cross section as small punctate areas of sclerosis, often called the polka-dot appearance. At MR imaging,
areas of trabecular thickening have low signal intensity, regardless of the pulse sequence used. On T1-weighted MR
images, the signal intensity of vertebral hemangiomas varies from low to high, depending on the degree of adipose
tissue present. T2-weighted MR images usually show areas of very high intensity corresponding to the vascular
components [49]. CT or MR images obtained after intravenous administration of contrast material demonstrate lesion

12. enhancement.
Vertebral hemangiomas occasionally cause neurologic symptoms from spinal cord compression, particularly if these
lesions extend into the posterior elements or surrounding soft tissues, expand and fracture bones. [44,45,46,47]. The
larger the degree of fat overgrowth in the stroma between thickened trabeculae (seen on CT images as low attenuation
between thickened trabeculae and as areas of high intensity on T1-weighted images and intermediate intensity on T2-
weighted images), the less likely these lesions will be symptomatic (inactive hemangioma) as shown by Laredo and
coworkers [50].
Calvarial hemangiomas account for 20% of all hemangiomas and are most frequent in the frontal or parietal region
[46]. These lesions arise in the diploic space and cause expansion that often involves the outer table to a greater extent.
At radiography and CT, a calvarial hemangioma commonly appears as a lytic lesion with a pattern of radiating,
weblike or spoke-wheel, trabecular thickening [44,45,46,47]. This characteristic appearance, as in vertebral lesions, is
caused by preexisting trabeculae that have become thickened through intramembranous bone formation adjacent to
the angiomatous channels. Recognition of the pattern should alert the radiologist to the vascular nature of the lesion.
Osseous hemangiomas in other locations may also have radiating trabecular thickening on radiographs. Another
common pattern is a bubbly bone lysis that creates a honeycomb, latticelike, or quot;hole-within-holequot; appearance. These
lytic areas are invariably multifocal and usually metaphyseal or epiphyseal. Bone lysis can have linear and circular
components on radiographs, suggestive of a vascular lesion, with linear and circular elements representing vascular
channels seen longitudinally and en face, respectively. However, these serpentine vascular channels are recognized
more easily with CT and MR imaging. Characteristically, these channels have low signal intensity on T1-weighted
images and very high signal intensity on T2-weighted images because of slow blood flow. In arteriovenous lesions with
faster blood flow, low signal intensity may persist with all MR imaging pulse sequences. The appearance of osseous
hemangiomas at bone and red blood cell labeled scintigraphy is variable, from photopenia to moderate increased
activity [51,52].
Periosteal or cortical hemangiomas occur most frequently in the anterior tibial diaphysis. These lytic cortical lesions
may also show the characteristic multifocal vascular channels or be seen as a larger, nonspecific region of bone
destruction. Cortical hemangiomas may predispose the bone to fracture, and periosteal reaction may accompany these
lesions.
 Addendum
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