Case ReportIntradural-Extramedullary Capillary Hemangioma with Acute Bleeding: Case Report and Literature Review
Introduction
Capillary hemangiomas are benign tumors. They appear frequently in mucosal soft tissues and the skin of head and neck in children.1 When they develop in the spine, they are located most frequently in the vertebral body. Extraosseus involvement of the spine is extremely rare. When they appear in the neuroaxis, they are usually intradural extremedullary lesions.
This kind of lesion is most frequently found in middle-aged males. The common clinical history is a long-lasting back pain that slowly progresses with radicular pain or neurologic deficit. Acute presentation is very rare. Despite hemangiomas of the spinal cord are rare, early diagnosis is important to avoid complications that would lead to neurological deterioration.2 We report a case of capillary hemangioma of the spinal cord that hemorrhaged and caused a sudden paraplegia.
A 58-year-old male presented to the emergency department complaining of upper lumbar pain of 1 week's duration that was triggered by mild trauma. The pain continued to worsen despite analgesic treatment, and 15 days later he returned to the emergency room reporting weakness and paresthesias in both legs, as well as urinary and fecal retention.
Urgent magnetic resonance imaging (MRI) of the spine revealed an 1-cm-long intradural-extramedullary lesion at the T10-11 level. The lesion was isointense to hyperintense on T1-weighted MRI (Figure 1) and isointense to mildly hyperintense on T2-weighted MRI. The lesion showed heterogeneous contrast enhancement. The spinal cord presented perilesional edema seen as hyperintensity on T2-weighted MRI. Based on these features, we assigned a differential diagnosis of meningioma, neurinoma, or a vascular intradural-extramedullary tumor.
Given the patient's sudden deterioration, urgent surgery was performed under neurophysiologic monitoring. A laminotomy at T10-11 and middle durotomy were performed. Opening the dura revealed a subdural subacute hemorrhage. The lesion was reddish and bleeding, and intimately attached to the posterior surface of the spinal cord. An en bloc resection was performed. Once hemostasia was accomplished, the wound closed by layers. Motor evoked potentials improved after the surgery (Figure 2).
The macroscopic aspect was brownish and of elastic consistency. Optic microscopy sections showed extensive hemorrhage within a benign proliferation of small vessels. The vessels had an irregular shape and thin capillary-like walls, without significant cellular atypia. These vessels stained positively with CD34 (diffuse in endothelial cells) and GLUT1 (in isolated cells). The neoplasm was negative for WT1, epithelial membrane antigen, glial fibrillary acidic protein, and S100 (Figure 2), leading to a diagnosis of capillary hemangioma (Figure 3).
The postoperative course was uneventful, and the patient improved from the previous deficits until he could walk with help at discharge. At an 18-month follow-up, the patient exhibited only mild gait alteration.
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Discussion
Spinal tumors account for approximately 15% of all central nervous system neoplasms, and vascular lesions comprise approximately 6%–7% of all spinal intradural tumors. These lesions include capillary telangiectasias, cavernous angiomas, and cavernous hemangiomas3, 4; the most frequently seen lesions in this group are cavernous hemangiomas and capillary hemangiomas.3 In the intradural extramedullary space, hemangiomas can arise from the blood vessels, root nerves, inner layer of the dura mater,
Conclusion
To the best of our knowledge, this is the first case of an intradural extramedullary capillary hemangioma presenting as acute neurologic deterioration due to intratumoral bleeding. Extramedullary intradural hemangioma is a rare lesion that may be clinically and radiologically indistinguishable from other lesions. Early treatment is important to achieve a good neurologic outcome.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.