Craniocervical junction intradural extramedullary meningioma with cord compression

Abstract

Craniocervical spine meningiomas are rare. They often present with non-specific motor or sensory symptoms. Presenting symptoms can include gait ataxia, radiculopathy, myelopathy, back pain and sensory deficits. Spinal meningiomas are slow-growing tumours, with an insidious onset. Due to the critical location of craniocervical meningiomas, severe symptoms such as respiratory distress and quadriparesis are possible. We describe the clinical presentation of a craniocervical junction meningioma, its relevant neuroimaging findings, diagnostic challenges and management. A woman in her 30s presented with a subacute onset of neck pain, headaches, paresthesia and a Hoffman’s sign of the left upper extremity. A cervical spine MRI revealed an intradural extramedullary craniocervical junction meningioma involving the C1 segment with cord compression. The tumour measured 1.4×2×2.2 cm. A mid-line suboccipital craniectomy, tumour resection (Simpson grade II) with cervical laminectomy, and dural grafting were completed for definitive management. A brief literature review was conducted yielding a total of 24 cases.

Background

Primary spinal cord tumours comprise only 2%–4% of all primary central nervous system tumours.¹ Spinal cord tumours are characterised by their anatomical location: intramedullary, intradural extramedullary and extradural. Intramedullary tumours are generated by ependymal cells or astrocytes and arise from the cord itself. Intradural extramedullary tumours arise from within the dura but outside of the spinal cord. These tumours are often meningiomas or nerve sheath tumours (ie, schwannoma). Extradural tumours arise outside the spinal cord or dura and usually occur due to metastatic disease. Extradural tumours are typically seen within the vertebral bodies secondary to metastasis and can cause pathological fractures. Intradural metastasis and spinal cord compression are rare but have been observed.² This case highlights the diagnostic challenge faced by neurologists when evaluating patients with non-specific sensory complaints. Neurological signs and symptoms of radiculopathy often go dismissed, however, such symptoms may be consistent with a life-threatening intradural neoplasm which may progress to permanent neurological dysfunction.

Case presentation

A woman in her 30s presented to the emergency department with a 2-week history of pain and paresthesia of the left upper extremities. Physical examination revealed mild loss of vibratory sensation in the left upper extremity both distally and proximally. In addition, the patient endorsed numbness and tingling when moving her wrist, elbow and shoulders. The musculoskeletal examination was benign. No dysmetria or hyper-reflexia was observed. There was no loss of proprioception. The patient was discharged with oral neuropathic analgesics and was scheduled for an outpatient MRI of the brain. The MRI of the brain revealed a heterogenous signal within the cervical cord and the patient was encouraged to present for revaluation. No lesions within the brain were identified. Her paresthesia had progressed and begun to involve the left side of the neck and face. Physical examination now revealed moderate loss of vibratory, pinprick and temperature sensation in the left upper extremity. A left-sided Hoffman’s reflex was now present. Symptoms were worse with the left lateral flexion of the neck. There was no dysmetria, and the Romberg test was negative.

Investigations

Serum testing with a complete blood count and comprehensive metabolic panel was unremarkable. Non-contrast head CT, MRI of the brain and head CT angiogram were completed revealing no acute intracranial abnormalities, which showed a possible high cervical spine tumour. MRI with and without gadolinium (Gd) contrast of the cervical spine confirmed an intradural extramedullary neoplasm involving the C1 segment of the cervical spine with cord compression (figure 1). The tumour measured 1.4×2×2.2 cm. The patient was diagnosed with a craniocervical junction (CCJ) intradural extramedullary meningioma.

Figure 1.

MRI of the cervical spine with gadolinium contrast enhancement. Non-contrast (A) and contrast-enhanced (B) sagittal sequence revealing intradural extramedullary neoplasm (arrow). (C) Axial contrast-enhanced sequence revealing intradural meningioma at C1 (arrow) with cord compression. (D) Gradient echo sequence revealing no microhaemorrhages (arrow).

Differential diagnosis

The initial neurological manifestations were consistent with radiculopathy that likely occurred due to a compressed nerve root. Given the patient’s young age and female gender multiple sclerosis was also part of the differential diagnosis however was given less consideration. The second neurological examination showed symptoms (ie, loss of vibratory sensation, Hoffman’s sign) localising to the cervical spinal cord, particularly the dorsal columns.

Treatment

Initial management included oral dexamethasone for cervical cord compression, and she was planning to undergo surgery. A standard midline incision was planned and marked from the external occipital protuberance down to the level of C2. A midline incision was opened using a 10-blade. Bovie electrocautery was used to dissect down to the subocciput and the C1–C2 spinous processes. Using a high-speed bur, a C1 and C2 laminectomy was performed. Using a 15-blade and Metzenbaum scissors, the dura was opened linearly in the midline from C2 through the suboccipital dura. Under the microscope, the spinal cord was identified to be rotated to the right side of the canal. The tumour was visible ventral to the spinal cord. The arachnoid was opened sharply, and the dentate ligament was sectioned. The left spinal accessory nerve was identified, freed from its arachnoid adhesions and mobilised medially. The tumour was then coagulated and entered sharply. Pathology was consistent with a WHO grade I meningioma. The tumour was sequentially debulked internally. It was then mobilised laterally into the window between C1 and C2 nerve rootlets. The tumour was resected in its entirety. The dural attachment was then coagulated and resected (Simpson grade II). Throughout the entire surgery, motor-evoked potentials and somatosensory-evoked potentials remained stable. The spinal dura was closed in a watertight fashion. The patient was observed postoperatively in the neurological intensive care unit. A repeat MRI of the cervical spine depicts successful tumour resection (figure 2).

Figure 2.

Repeat MRI of the cervical spine depicts successful tumour resection (arrow).

Outcome and follow-up

A repeat MRI of the cervical spine depicts successful tumour resection (figure 2). At the 1-month follow-up appointment, the patient’s symptoms had significantly improved with mild intermittent headache. At a 3-month follow-up appointment, the patient continued to remain symptom-free with no residual side effects.

Discussion

We presented the case of a woman in her 30s with an insidious onset of non-specific sensory findings of the bilateral upper extremities eventually found to have a CCJ meningioma. CCJ meningiomas are a rare subset of spinal cord neoplasms. Given their dense anatomical location, they often lead to cord compression. Spinal meningiomas are commonly seen within the thoracic and cervical spine. These neoplasms are less common in the lumbar spine and seldom reported in the sacrum. Craniocervical neoplasms arise from the clivus and C2 lamina. Craniocervical meningiomas may be anterior to the neuraxis (ventral to the dentate ligament), as seen in our case, or posterior to the neuraxis (dorsal to the dentate ligament).³

Symptomatology can vary widely based on the specific structures damaged across the neuraxis and follow a gradual, insidious process, which is evident in the patient described here. Her symptoms were initially classified as peripheral neuropathy. However, symptoms did not resolve with neuropathic analgesics and progressed, leading to her second presentation. These discrepancies can be adequately managed through careful interpretation of the anamnesis and utilisation of neurodiagnostic tests (ie, CT or MRI of the cervical spine). The Hoffman reflex represents an upper-motor neuron sign likely from the cord compression demonstrated on neuroimaging. The patient’s altered sensory examination was mainly in the vibratory modality, representing a lesion to that dorsal column (posterior cord). However, neuroimaging revealed primary anterolateral involvement of the neoplasm anterior to the dentate ligament. The vibratory sensation is difficult to localise and sometimes can arise from the spinothalamic tracts. This case highlights the diagnostic challenges that can arise with these constellations of symptoms.

Surgical resection provides definitive management in conjunction with surveillance of recurrence.⁴ The Simpson grade was first described in 1957 and correlated surgical resection completeness with the rate of recurrence (figure 3). Difficulties achieving Simpson grade I, when the tumour engulfs nearby neurovascular structures, can arise. In some cases, radiation therapy is used in conjunction with surgical management. Although widely used, the Simpson grading scale has been scrutinised due to its subjectivity in intraoperative visualisation.⁵ The far lateral transcondylar approach (also known as extreme lateral infrajugular transcondylar approach) is a surgical technique used to access lesions located at the craniovertebral junction and the upper cervical spine, such as anteriorly located CCJ meningiomas. This approach provides direct access to anteriorly located tumours at the CCJ, allowing surgeons to remove these lesions while minimising manipulation of the neural structures.⁶ The far-lateral transcondylar approach allows for the preservation of the vertebral artery in most cases, which is critical to avoid potential ischaemic complications.⁷

Figure 3.

Simpson grade of meningioma resection.

A brief literature review was conducted using PubMed. The search term “cranio-cervical junction meningioma” was used, and the case report/series filter was used. The results are described in table 1.^8–11

Table 1.

Summary of cases from the literature review

Reference	Study type	Sample size	Age	Gender	Symptoms	Imaging	Surgical technique	Outcomes
Das8	Case report	1	45	F	Quadriparesis with diffuse hyperreflexia.	MRI: Craniocervical meningioma with cord compression.	Midline suboccipital craniectomy	Simpson grade I tumour resection with C1-2 laminectomy. Significant improvement in symptoms at 2-month follow-up
Abolfotoh et al9	Case report	1	37	F	5 years of progressive right upper extremity numbness and weakness, right facial numbness, and occipital pain.	MRI: Large calcified mass at the right posterior-lateral margin of the craniocervical junction, encasing the right vertebral artery and right PICA loop	Resection of the tumour was done using the transcondylar approach	Successful resection with no recurrence at 14 months postoperatively.
Lilla et al10	Case Series	4	Mean: 65	F (4)	Head and neck pain (1). Paresthesia (1). Gait ataxia (1). Recurrent syncope (1).	Tumours were located from the clivus to C2.	Resection of anteriorly located upper cervical and lower clivus meningiomas without laminotomy or craniotomy using a minimally invasive posterior approach	Simpson grade II tumour resection. No tumour reccurence or spinal instability at the 7–28 months follow-up period.
Dührsen et al11	Retrospective cohort	18	Mean:52	F (14) M (4)	Pain (3). Sensory/motor disability (11). Lower CN palsy (3). Incidental (1).	Tumours were located between C0 and C2 in ten patients, at the level of the foramen magnum in three patients, and at the level of clivus, extending caudally in five patients.	Retrosigmoidal (1), suboccipital midline (11), suboccipital midline with lateral extension (6), with additional laminectomy of C1.	Simpson grade II (16), Simpson grade III (2). At follow-up one patient had two reccurences at one and 3 years postoperatively.

PICA, Posterior inferior cerebellar artery.

Learning points.

• An intradural tumour at the craniocervical junction can present with non-specific neurological symptoms (eg, paresthesia).

• Consideration for further neurological imaging including an MRI of the cervical spine should be given consideration.

• In the inpatient setting, the neuro hospitalist should consider a CT scan of the cervical spine before patient discharge.

• Craniocervical junction meningiomas are most often managed using the midline suboccipital approach.

• A minimally invasive posterior approach may leading to less pain, faster recovery, lower risk of complications and less scarring.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).