Abstract

Background:Extradural hemangiomas are rare, have varied and challenging clinical presentations, and require special considerations from the management point of view.

Case Description:A 70-year-old female presented with back pain that was ultimately attributed to a thoracolumbar extra-dural “dumbbell” hemangioma. Following surgical resection, the patient did well.

Conclusion:Extradural hemangiomas may present as spinal extradural soft tissue masses that must be differentiated from dumbbell neurofibroma.

Keywords: Extradural hemangioma, nerve sheath tumor, thoracolumbar spine

INTRODUCTION

Hemangiomas are congenital vascular malformations of unknown etiology.[ 1 5 ] In the spine, vertebral body hemangiomas are some of the most commonly reported lesions.[ 5 ] Extradural hemangiomas are rare and only infrequently present without osseous involvement.[ 2 4 5 ] A lumbar extradural “dumbbell” hemangioma requires differentiation from a dumbbell nerve sheath tumor and appropriate excision for optimum clinical outcomes.

CASE REPORT

Clinical presentation and magnetic resonance/computed tomography findings

A 70-year-old Chinese female presented with deep-seated epigastric pain radiating to the back/flanks without a focal neurological deficit. The esophagogastroduodenoscopy was negative. The patient underwent computed tomography (CT) scan of the abdomen/pelvis, which documented an enhancing extradural spinal lesion measuring approximately 2.3 × 1.5 cm at the level of the L1 and L2 vertebrae, resulting in severe central canal stenosis [ Figure 1 ]. It extended through the left L1/L2 foramen and into the left paravertebral region, abutting the left psoas muscle. Although there was no bony erosion/destruction, the L1 vertebral body showed posterior scalloping abutting the lesion. A supplemental magnetic resonance imaging (MRI) scan scan confirmed an enhancing “dumbbell shaped” 4.8 × 3.9 × 1.4 cm (CC × TV × AP) intraspinal extradural lesion centred at the L1/L2 level [ Figure 2 ]. It was isointense on T1, hyperintense on T2, and homogeneously enhanced with contrast. On MRI, it resulted in severe L1/L2 spinal stenosis, compressing the conus/filum terminale, and extending into the left L1/L2 neural foramen. Based on the CT/MR images, the differential diagnoses included schwannoma, neurofibroma, lymphoma, and metastasis.

Figure 1

CT scan of Thoraco-lumbar spine showing space occupying lesion on spinal canal (Pre-op)

Figure 2

Contrast enhanced MRI images of thoraco-lumbar spine showing dumbbell shaped tumor in spinal canal

Surgery

The patient underwent T12–L3 laminectomy with L1–L2 left facetectomy for excision of the tumor accompanied by T12–L3 instrumented fusion [ Figure 3 ]. Intraoperatively, a completely extradural highly vascular soft-tissue lesion was found at the L1–L3 vertebral levels. Intraoperative frozen sections showed fibroadipose tissue with numerous vascular channels closely associated with arterioles and venules. The findings were suggestive of a vascular malformation. The patient recovered well postoperatively.

Figure 3

Post-op xray of thoraco-lumbar spine after decompression and posterior stabilisation

Histology

Histological examination showed fibroadipose tissue with diffuse proliferation of densely packed vascular channels closely associated with some arterioles and venules without any thrombosis [ Figure 4 ]. Immunohistochemical staining found the endothelial cells to be CD31 immune-reactive [ Figure 5 ]. The rare lymphatics channels were immune-reactive to D2-40 stain. However, the stains for Melan-A and HMB-45 were negative.

Figure 4

Histopathological slide showing vascular lesion composed of capillaries, arterioles and venules with intercepting adipocytes

Figure 5

Immunostaining slide showing CD31 stained endothelial cells within the vessels

Postoperative course

Four months after the surgery, the patient remained neurologically intact. MRI repeated 3 months postoperatively showed complete resection of tumor and no cord compression [ Figure 6 ].

Figure 6

MRI images at post-op 3 months showing complete resection of tumor without any signs of recurrence

DISCUSSION

Extradural hemangiomas are rare, and are characteristically slowly progressive lesions that rarely present with acute neurological deterioration.[ 3 5 ] CT scan and MRI studies identify extradural hemangiomas as hyperdense on CT, isointense on T1, hyperintense on T2 weighted MR images, and homogeneous enhancement with contrast.[ 3 ] They are typically round or ovoid in shape, tend to extend through intervertebral foramina, and are usually located in the ventral extradural space of the lumbar spine.[ 3 ] Histologically, they are soft-tissue vascular malformations (e.g., abnormal arteries and veins) that frequently demonstrate degenerative changes due to repeated thrombosis and fibrosis.[ 5 ]

The most common differential diagnoses include schwannoma, lymphoma, meningioma, angiolipoma, disk herniation, synovial cysts, granulomatous infection, pure epidural hematoma, and extramedullary haematopoiesis.[ 2 3 4 5 ] Complete surgical resection of hemangioma may be complicated by copious intraoperative hemorrhage and the possibility of incomplete tumor resection.[ 2 3 4 5 ] Re-operation for residual/recurrent tumors is associated with higher complication rates and poorer outcomes.[ 3 4 ] This case highlights unique problems with the diagnosis and management of spinal hemangiomas and special considerations to be kept in mind while dealing with these rare lesions.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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