Intradural extramedullary cervical cavernoma

Abstract

Background:

Spinal cavernomas (SCs) account for about 5% of all spinal vascular malformations. Intradural SCs occur in just 3% of cases and are typically intramedullary.

Case Description:

A 58-year-old female presented with progressive left occipital neuralgia, left cervicobrachial neuralgia, and paresthesia of all four extremities. The magnetic resonance imaging (MRI) revealed an intradural extramedullary C2–C4 lesion causing significant spinal cord compression. Gross total tumor excision was accomplished through a midline laminectomy pathologically; the lesion proved to be a cavernoma. The postoperative follow-up MRI obtained 4 months postoperatively showed complete tumor resection.

Conclusion:

A 58-year-old female successfully underwent gross total excision of a C2–C4 intradural extramedullary SC.

INTRODUCTION

Cavernomas, also called cavernous malformations or cavernous angiomas, are rare developmental low-flow vascular malformations.[7,18] Although spinal cavernomas (SCs) comprise 5% of all spinal vascular malformations, only 3% are intradural and extramedullary lesions. According to Ismaiel et al.,[7] only 41 cases of intradural extramedullary cavernomas have been published to date. Here, a 58-year-old female with a cervical intradural extramedullary C2–C4 cavernoma successfully excised through a laminectomy.

CASE PRESENTATION

A 58-year-old female presented with progressive left occipital neuralgia for 3 months, left non-systematized cervicobrachial neuralgia for 2 months, and paresthesia of all four extremities for 1 month. She had mild cervical pain and hyperactive reflexes but was otherwise neurologically intact. The cervical magnetic resonance imaging (MRI) [Figure 1] showed an intradural extramedullary anterior/left sided C2–C4 heterogeneous mass (i.e., 12 × 16 mm) markedly compressing the cord. It was hyperintense on T1-weighted imaging (WI) and hypointense on T2-WI.

Figure 1:

(a) Sagittal and (b) axial sections of a cervical spine magnetic resonance imaging on T2-weighted imaging (a and b) showing an anterior intradural extramedullary lesion at the level of C2–C4.

Surgery

Through a laminectomy of C2, C3, and C4 and durotomy, a dense, non-hemorrhagic tumor was encountered that was readily removed; there was a clear plane between the cord and the nerve roots. Pathologically, the tumor proved to be a cavernoma [Figure 2]. One month postoperatively, she had complete relief of her occipital and cervicobrachial neuralgia and exhibited only mild residual left upper arm paresthesias. The follow-up spinal MRI [Figure 3] performed 4 months postoperatively confirmed total lesion excision.

Figure 2:

The pathologic examination at HE (Hematoxylin and Eosin) stain at ×200 magnification reveals a notable proliferation of blood vessels. These vessels exhibit dilated lumens, which are prominently filled with red blood cells, indicating vascular congestion. The walls of these vessels are thickened and fibrous, suggesting a chronic process or significant fibrosis.

Figure 3:

Sagittal (a) and axial (b) sections of a cervical spine magnetic resonance imaging on T2-weighted imaging (WI) (a) and T1-WI (b) showing a complete removal of the cavernoma.

DISCUSSION

SCs are rare, representing between 5% and 12% of all spinal vascular anomalies.[2,7] Notably, only 3% are located intradurally, and most are located intramedullary. Of the roughly 40 cases of intradural extramedullary cavernomas reported in the literature, we were able to identify ten cervical lesions[3] [Table 1]. Intradural extramedullary cavernomas are most commonly encountered in the lumbar region, followed by the thoracolumbar junction, lower thoracic region, and cervical region.[7,8] Very rarely, the cavernomas precipitate the sudden onset of paraplegia due to an acute hemorrhage.[11,15]

Table 1:

Summary of literature for cervical intradural extramedullary cavernoma.

MR Diagnostic study of choice

MRI is the study of choice for diagnosing intradural extramedullary cavernomas.[3] They are defined by a reticulated core of mixed intensity, which can be associated with calcifications or dense fibrocartilage.[7] Cavernous angiomas often present with a surrounding rim of decreased signal intensity on T2-WI.[3] Notably, spinal angiography is not particularly helpful in diagnosing SC.[2,16]

Treatment

Cavernomas are benign lesions, and the optimal treatment is complete surgical removal, typically through laminectomies; often, there are clear planes between cavernomas and the surrounding neural structures[9,18] [Table 1]. They are typically slow-growing, but acute intralesional hemorrhage may lead to precipitous neural compression, warranting emergent surgical intervention.[3,15]

CONCLUSION

A 58-year-old female successfully underwent gross total excision of a C2–C4 intradural extramedullary SC.

Ethical approval

The Institutional review board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

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