Introduction

Meningiomas are among the most common benign tumours of the spinal canal in adults,1 found predominantly in the thoracic segments (80%). Clear cell meningioma is an aggressive variant with a predilection for the cauda equina.2 Magnetic resonance imaging (MRI) can show an intradural-extramedullary location.1 Gadolinium enhancement helps in the diagnosis.

According to the literature, the occurrence of an intramedullary cervical meningioma is very rare, with only five cases reported in the literature so far.1, 2, 3 We present the sixth case and review the previous reports.

Case report

A 21-year-old man presented with gradual numbness and parenthesis in the four limbs, particularly in the left leg and progressive difficulty in walking over a 2-year period. Physical examination detected spastic paraparesis, hyper-reflexia, impaired position and vibration senses, clonus, extensor plantar reflexes, and spastic gait MRI showed an intramedullary mass in the cervical cord with perifocal oedema, extending from the cervicomedullary junction to C2 (Figure 1). The tumour was isointense on T1 and iso- to hyperintense onT2-weighted images (Figure 1). Homogeneous enhancement with gadolinium on T1-weighted images was evident (Figure 2).

Figure 1
figure 1

Sagittal T2-weighted (a) and T1-weighted (b) MRI of the cervical cord reveals isointense and iso- to hyperintense intramedullary tumour at the level of C1 and C2. Tumour-related expansion of the cord and a developed syringomyelia are also evident. Note oedema of the cord at the distance between the tumour and the syrinx, which is apparent on T2-weighted MRI (a). Sagittal T2-weighted MRI (c) of the cervicomedullary showing tumour extension up to the cervicomedullary junction and associated oedema. MRI, magnetic resonance imaging.

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Figure 2
figure 2

Post-contrast sagittal T1-weighted MRI with gadolinium. Note the relatively homogenous enhancement of intramedullary tumour. MRI, magnetic resonance imaging.

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The tumour was subtotally resected through suboccipital craniotomy and C1–C2 laminectomy, applying a Cavitron ultrasound surgical aspirator under operating microscopy.

Histopathological examination confirmed a syncytial-type meningioma (World Health Organization Grade I). Immunohistochemical staining was positive for epithelial membrane antigen and S-100 protein (Figure 3).

Figure 3
figure 3

Photomicrographs of the tumour showing haematoxylin and eosin staining of a syncytial meningioma (a and b). Epithelial membrane antigen (c) and S-100 protein (d) staining.

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Discussion

To date, only five documented intramedullary spinal meningioma in the cervical cord have been described in the literature (Table 1). An age range from 22 months to 67 years has been reported (Table 1). Possible risk factors may be trauma, viral infections, irradiation or genetic mutations.

Table 1 Intramedullary cervical cord meningioma: concepts and review of reported cases
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Three of the five previously described patients were female. With this patient, sex ratio will become equal but with a predilection for female gender during the fifth to seventh decades of life (Table 1).

The insidious onset with neck pain, dysesthesia, motor deficits and urinary retention has been described. Our patient suffered from simultaneous sensory and motor deficits.

The tumour is more common in the upper cervical levels (Table 1). Moriuchi et al.3 reported a case of intramedullary meningioma with extension beyond the cervicomedullary junction into the medulla oblongata. This makes total resection less probable.

The subtypes of intramedullary cervical meningioma included fibroblastic,1 clear cell,2 transitional,3 angioblastic4 and papillary meningioma.5 Our case is the first meningothelial intramedullary meningioma in the cervical cord.

Clear cell and angioblastic subtypes have been associated with a less favourable outcome (Table 1). Jallo et al.2 recommend treating these tumours aggressively with MRI follow-up every 3–6 months during the first years and annually, thereafter. Concurrent involvement of the medulla oblongata contributes to a less favourable outcome.

After 3 years, our patient shows no recurrence, but long-term follow-up has to be awaited. Imaging characteristics of intramedullary meningioma have not been well described and only two previous patients had spinal MRI. Salvati et al.1 do not mention MRI specifications in their two cases. Moriuchi et al.3 showed spinal cord expansion and homogeneously enhanced intramedullary tumour extending up to the medulla oblongata. Our case provides additional data on imaging of these rare tumours in the cervical region (Figures 1, 2 and 3).

Conclusion

Intramedullary cervical meningioma is a very rare clinical entity. When confronting a homogenously enhanced intramedullary tumour of the cervical cord on post-contrast MRI, meningioma should be included in the differential diagnoses. Because of high recurrence after surgery and aggressive behaviour of some variants, meticulous histopathological examination is mandatory to predict evolution and plan follow-up. Total resection of the tumour is the treatment of choice.