Capillary haemangioma of the spinal cord: Report of two cases and a review of literature
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.263193
Source of Support: None, Conflict of Interest: None
Haemangiomas are benign vascular tumours. Haemangiomas in the spinal region are rare lesions, which mostly occur in the intradural extramedullary compartment; and, the occurrence of epidural capillary haemangiomas is exceedingly rare. Histologically, cavernous haemangiomais are the most common spinal haemangiomas, and very few cases of spinal cord capillary haemangioma have been documented., To the best of our knowledge, only 14 patients with an epidural capillary haemangiomas in the spinal canal have been reported in the literature till date.,,,, As these tumours are very rare, not much is known about their natural history.
We report two rare cases of spinal haemangiomas, one of them purely being in the epidural extramedullary compartment, and the other in the intradural extramedullary compartment.
A 51-year old male patient presented with complaints of radicular pain in the right lower limb for 2-3 months. The previous medical history and family history were unremarkable. On general physical examination, no significant abnormality was detected.
Magnetic resonance imaging (MRI) of the spinal cord revealed a contrast enhancing extradural lesion in the right lumbar L1-L2 region. The clinical possibilities of a nerve sheath tumour or a metastatic tumour were considered.
The tumour was completely excised and sent for histopathological examination. The intraoperative finding was a well-defined, moderately vascular, extradural tumour at L1-L2 level. The exiting nerve root was compressed by the lesion but the tumour was separate from its sheath. Erosion of the posterolateral wall of L1 vertebral body was present because of chronic compression. Complete excision of the tumour was achieved.
Histological examination revealed a well-circumscribed, lobulated tumour, which was composed of small, capillary-sized vascular channels. These vascular channels were thin-walled and lined by a single layer of endothelial cells. Some vascular spaces were larger and showed a thickened wall. No atypia or mitosis was seen. The endothelial cells were highlighted by cluster of differentiation (CD)31 and CD34. The stroma showed oedema and a mild myxoid change [Figure 1].
A 48-year old male patient presented with complaints of radicular pain in the left lower limb since 2-3 months. The previous medical history and family history were unremarkable. MRI of the spinal cord revealed a well-circumscribed contrast enhancing intradural extramedullary lesion in the D12-L1 region. The clinical possibilities of a meningioma or a neurofibroma were considered.
Intraoperatively, it was a well-circumscribed, moderately vascular, intradural tumour. It was not entrapping any nerve root. The tumour was completely excised and sent for histopathological examination.
On microscopic examination, a lobulated tumour was seen, which was composed of numerous variable capillary-sized vascular spaces, which were thin-walled and lined by a single layer of plump endothelial cells. The lobules were separated by fibrous septae. No atypia or mitosis was seen. The stroma showed oedema. The endothelial cells showed positivity for vascular markers such as CD31 and CD34 [Figure 2].
At follow up, both the patients are symptom-free and doing well 6 months after the surgery.
Haemangiomas are ubiquitous hamartomatous malformations that result from proliferation of vascular endothelial cells. These lesions are classified according to the predominant type of vascular channel (i.e., capillary, cavernous, arterio-venous, or venous) that is observed upon histological examination. These haemangiomas are most commonly found in the skin, soft tissues, and bone, and they typically regress without surgical treatment.
Spinal cord tumours comprise about 15% of all central nervous system (CNS) neoplasms. Of these, 2%–7% are of vascular origin. Among the vascular lesions of the spinal cord, cavernous haemangioma is the most common lesion. Capillary haemangiomas in the spinal cord are rare and only a few cases have been reported. Most of the spinal haemangiomas have been reported in the vertebrae. Rarely, they arise from the spinal cord. The most frequent sites of these tumours are around the cauda equina and conus. Spinal epidural haemangiomas account for 4% of all spinal epidural tumours, mostly occurring as a primary lesion in the vertebral bone; however, they can also occur at other locations., In the spinal intradural extramedullary space, haemangiomas may arise from the blood vessels of the nerve roots in the cauda equina, the inner surface of the dura, or the pial surface of the spinal cord. The occurrence of a purely spinal epidural haemangioma is exceedingly rare, and most of the reported cases were of the cavernous type. Spinal capillary haemangiomas can arise from any compartment, although they are found most commonly in the intradural extramedullary location. Epidural capillary haemangioma of the spinal cord is exceedingly rare. To the best of our knowledge, only 14 cases of epidural capillary haemangiomas in the spinal canal have been reported in the literature till date.,,,, Ten of these cases involved the thoracic spine ,,, and two cases involved the lumbar level., In our case, both the cases involved the lumbar spine.
Capillary haemangiomas of the spinal cord present in a chronic progressive manner because of mass effect and nerve root irritation whereas cavernous haemangiomas present with an acute symptomatology related to intratumoural bleeding. All reported cases of capillary haemangiomas have presented with back pain, radicular pain, or chronic myelopathy due to mass effect.
Most capillary haemangiomas of the spinal cord are radiologically misdiagnosed and/or mistaken for meningiomas or schwannomas., Some reports have described spinal haemangiomas as dumbbell-shaped lesions., However, none of our cases were dumbbell shaped on radiological examination. The differential diagnosis for these lesions includes a nerve sheath tumour, meningioma, haemangiopericytoma, haemangioblastoma, cavernous haemangioma, metastatic carcinoma and lymphoma. Capillary haemangiomas of the spinal cord and proximal nerves must be distinguished from haemangioblastomas. Haemangioblastomas are most commonly located in the posterior fossa, but up to 5% occur in the spinal cord. They contain stromal foam cells in addition to a rich capillary network.
The treatment of choice for capillary haemangiomas is complete surgical removal. Gross total resection should be the goal., The prognosis is typically good, and no relapses have been reported in the literature.
Spinal capillary haemangiomas are very rare but they should be included in the differential diagnosis of a spinal mass. They usually present with progressive myelopathy. On MRI, they appear as contrast enhancing lesions in the intradural or epidural space. They are benign and potentially curable so an early treatment may prevent the occurrence of a neurological deficit. Clinically, they may mimic other diseases such as a metastatic carcinoma, nerve sheath tumour or meningioma based on location, so an accurate histological diagnosis is essential for adequate management.
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