Spinal cord tumors
Primary spinal cord tumors are intramedullary (ependymoma, astrocytoma, hemangioblastoma) or intradural extramedullary (meningioma, schwannoma, neurofibroma) masses that cause a slowly progressive myelopathy; radicular pain heralds an extramedullary tumor, while painless dissociated sensory loss with sacral sparing points to an intramedullary one. Gadolinium-enhanced MRI of the affected cord level localizes the tumor, tissue from resection confirms it, and maximal safe surgical resection is the mainstay of treatment, with radiotherapy for residual or high-grade disease.
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What this article covers
- Overview
Definition: primary spinal cord tumors are neoplasms arising within the dura, either inside the cord parenchyma (intramedullary) or between the dura and the cord (intradural extramedullary), producing myelopathy, radiculopathy, or both.
- Epidemiology
Relative frequency: metastatic tumor is the most common spinal tumor overall, and benign spinal cord tumors occur more often than primary malignant ones.
- Risk factors
NF2-related schwannomatosis: the strongest risk factor; an autosomal dominant NF2 (merlin) mutation on chromosome 22 predisposes to spinal ependymomas, schwannomas, and meningiomas.
- Pathophysiology
Cell of origin determines the compartment: ependymomas arise from the ependymal cells lining the ventricular system and spinal canal, astrocytomas from astrocytes, hemangioblastomas from…
- Clinical features
Progressive myelopathy is the hallmark: upper motor neuron weakness, spasticity, hyperreflexia, and extensor plantar responses with sensory loss at or below the lesion; a sensory level on ascending…
- Differential diagnosis
Malignant spinal cord compression: known cancer, pain worse with recumbency preceding deficits by weeks, and an extradural mass from a destroyed vertebral body on MRI.
- Diagnosis
Diagnosis is established by contrast-enhanced MRI showing an enhancing mass that is assigned to its compartment (intramedullary, intradural extramedullary, or extradural), and confirmed by…
- Management
First, if deficits are acute or progressing (new weakness, sphincter dysfunction, rising sensory level): request urgent neurosurgical evaluation and start dexamethasone 10 mg IV once, then 4 mg IV or…
- Complications
Permanent paraparesis or quadriparesis: full neurologic function is rarely regained once compression has produced a fixed deficit.
- Prognosis
Benign extramedullary tumors carry a good prognosis: complete resection of a meningioma or schwannoma is typically curative.
- Prevention
No primary prevention exists for sporadic tumors; prevention means surveillance of predisposed patients.
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