Key-words:
Cavernous angioma of the spine - epidural spine tumor - magnetic resonance imaging
spine - spinal tumor - spine tumor surgery - vascular spine lesion
Introduction
Cavernous angiomas are common lesions of the central nervous system characterized
by abnormally dilated blood vessels lined by a thin endothelium. In the spine, a common
location is the vertebral bodies. Intradural extramedullary and intramedullary cavernous
hemangiomas are less frequent lesions, while purely epidural locations are uncommon.[[1]] Epidural cavernous angiomas represent 12% of spinal axis cavernous malformations.[[2]]
The earliest case was reported in 1978 by Decker et al.[[3]] They are indistinguishable from that of a schwannoma, which is a much more common
lesion. Here, we report a case of thoracic epidural cavernous angioma who presented
with chronic backache.
Discussion
Cavernous angiomas of the spine are benign and are vascular hamartomas representing
a dysplasia of the vessel-forming mesoderm. Harrison et al. postulated that primordial
vessels may lose their capacity to differentiate, resulting in a cavernous malformation.[[4]]
Microscopically, cavernous malformations are composed of closely opposed sinusoidal
vascular spaces. The walls consist of an innermost single layer of endothelial cells
surrounded by adipose tissue; elastic fibers or smooth muscle cells are absent.[[5]]
We reviewed the literature of 46 case reports (including our case). The first case
we found was reported by Decker et al. in 1978 [[Table 1]].
Table 1: Review of literature
Table 1: Contd...
The average age of patients was 50 years. The youngest was 13 years old and eldest
was 79 years old. There were 27 males and 19 females with slight male dominance.
Clinical symptoms were suggestive of the spinal cord or nerve root compression. Pain
was the most common symptom. Cases presented as neck pain, interscapular pain, or
back pain depending on the location of the lesion. They were also associated with
numbness and radicular pain along the dermatomes of involved roots. Twenty cases presented
with spastic paraparesis and urinary frequency or retention.
Thoracic spinal cord was the most common location. In 27 cases, thoracic spinal cord
was involved. In our case, dorsal D7 and 8 epidural space was involved. The cervical
spine was involved in 6 cases. The lumbar spine was involved in 11 cases. The sacral
epidural space was involved in 1 case.
Radiological features
MRI finding showed an epidural lesion of varying sizes. It is hyperintense on T2-weighted
image and isointense on T1-weighted image. Homogeneous contrast enhancement was seen
with gadolinium administration. The lesion was completely extradural but tightly adherent
to the dura mater. Hemorrhage was seen only in one case reported by Khalatbar et al.[[21]]
Dumbbell-shaped, extraforaminal extension was seen in more than 10 cases. These features
resemble MRI findings of schwannoma.[[23]] Therefore, it is difficult to diagnose on radiological imaging alone.
Surgery
All patients underwent surgery either laminotomy or laminectomy. The tumor was extradural,
purple or mulberry like, soft, and highly vascular. All surgeries were planned except
few. An emergency surgery was performed for hemorrhage into the lesion in one case
reported by Khalatbar et al. Li et al. reported four cases where surgery was done
on an emergency basis for sudden onset of paraparesis and urinary retention. The pathology
for sudden neurological deterioration was explained by Lee et al.
Cavernous angiomas do not grow by mitotic activity but have the propensity to enlarge
by thrombosis and bleeding, causing a spectrum of neurological syndromes ranging from
radiculopathy to sudden spinal cord dysfunction.[[24]]
From the surgical point of view, it is very important to understand that the extra-axial
cavernous angiomas behave like tumors and not like vascular malformations.[[19]]
Surgical decompression is the mainstay of treatment. Radiotherapy was not given as
a primary mode of treatment. Only in two cases, adjuvant radiotherapy was given few
months after surgery as symptoms persisted or worsened (reported by Sohn et al. and
Padovani et al.).
Fukushima et al. reported recurrence of the lesion in his case. He operated thrice
over a period of two decades.
Outcome
Spinal epidural angioma has a favorable outcome with total or subtotal removal. Complete
recovery was noted in 19 patients. Partial recovery was seen in 8 patients. Our case
had right knee extensor weakness in the postoperative period. He recovered within
2 weeks with no neurological deficits.
Conclusion
This observation and a review of the literature highlights that spinal epidural cavernous
angioma presents as compressive spinal cord pathology with symptoms of backache, weakness
in legs, difficulty in walking, or retention of urine. MRI spine with contrast helps
in preoperative diagnosis of angioma. It may present with sudden neurological deficits
due to hemorrhage which warrants emergency decompression. Surgical decompression is
the mainstay of treatment. There is no role of adjuvant radiotherapy. As the tumor
is highly vascular, hemostatic agents are required during surgery. The tumor may extend
into paravertebral space through the intervertebral canal. This may require additional
thoracotomy for tumor removal. Clinical recovery is complete after surgery in most
of the cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
