Spinal tumors may develop within the spinal cord parenchyma, directly destroying tissue, or outside the cord parenchyma, often compressing the cord or nerve roots. Symptoms can include progressive back pain and neurologic deficits referable to the spinal cord or spinal nerve roots. Diagnosis is by MRI. Treatment may include glucocorticoids, surgical excision, and radiation therapy.
(See also Overview of Spinal Cord Disorders.)
Spinal tumors may be intramedullary (within the cord parenchyma) or extramedullary (outside the parenchyma).
Spinal tumors may also be characterized as:
Primary: Originating in the spine
Secondary: Originating elsewhere in the body
Intramedullary tumors
The most common intramedullary tumors are gliomas, particularly ependymomas and low-grade astrocytomas. Intramedullary tumors infiltrate the parenchyma and expand rather than displace the spinal cord. Intramedullary tumors may extend over multiple spinal cord segments and obstruct the central canal within the cord, thus obstructing flow of the cerebrospinal fluid resulting in a peritumoral syrinx.
Extramedullary tumors
Extramedullary tumors may be
Intradural: Located within the dura but outside the spinal cord, rather than within its parenchyma
Extradural: Located outside the dura
Most intradural tumors are benign and are usually meningiomas, schwannomas, neurofibromas, or myxopapillary ependymomas (1). Myxopapillary ependymomas occur most often in the cauda equina (1).
Most extradural tumors are metastatic, originating as carcinomas of the lungs, breasts, prostate, kidneys, or thyroid. Other extramedullary tumors may be sarcomas, hematologic malignancies (such as multiple myeloma or lymphoma) or primary bone tumors. Primary bone tumors can be benign or malignant. Benign tumors such as osteoid osteoma or osteoblastoma are more common in children. Malignant tumors include chordoma, chondrosarcoma, Ewing's sarcoma, and osteogenic sarcoma.
Both intradural and extradural tumors exert their effects by compressing the spinal cord and its roots, rather than by invading the parenchyma. Most extradural tumors invade and destroy bone before compressing the spinal cord.
General reference
1. Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: Radiologic-pathologic correlation. Radiographics. 39 (2):468–490, 2019. doi: 10.1148/rg.2019180200
Symptoms and Signs of Spinal Tumors
Spine tumors manifest with 3 defined pain syndromes:
Biologic (so-called)
Mechanical
Radiculopathy (radicular)
Biologic pain is typically the earliest symptom of all spinal tumors. The pain usually occurs at night or early in the morning and resolves with activity. It is thought to be caused by inflammatory mediators secreted by the tumor, which are influenced by the diurnal variation in endogenous corticosteroids secreted by the adrenal glands or by administration of exogenous glucocorticoids.
Mechanical pain is caused by a spinal fracture and is worsened by movement or activity.
Radiculopathy (radicular pain) results from nerve root compression and follows the distribution of a nerve root.
Intradural extramedullary tumors often present with biologic pain but can also manifest radicular pain symptoms when the nerve roots are compressed.
Neurologic deficits referable to the affected level of the spinal cord may develop. Common examples are spastic weakness, incontinence, and dysfunction of the sensory tracts at a particular level of the spinal cord and below. Deficits are usually bilateral.
Some patients with intradural tumors—most often meningiomas and schwannomas—present with sensory deficits of the distal lower extremities, segmental neurologic deficits, symptoms of spinal cord compression, or some combination.
Symptoms of spinal cord compression can worsen rapidly and result in paraplegia and loss of bowel and bladder control.
Symptoms of nerve root compression are also common; they include pain and paresthesias followed by sensory loss, muscular weakness, and, if compression is chronic, muscle wasting, which occurs along the distribution of the affected roots.
Diagnosis of Spinal Tumors
MRI
Sometimes biopsy
Patients with segmental neurologic deficits or suspected spinal cord compression require emergency diagnosis and treatment.
The following symptoms and signs may suggest spinal tumors:
Progressive, unexplained, or nocturnal back (ie, biologic) or radicular pain
Segmental neurologic deficits
Unexplained neurologic deficits referable to the spinal cord or nerve roots
Unexplained back pain in patients with a history of cancer
This sagittal T2-weighted MRI scan shows a T8-T9 intramedullary ependymoma with syrinx.
This sagittal T2-weighted MRI scan shows a T8-T9 intramedullary ependymoma with syrinx.
Mark H. Bilsky, MD
This sagittal T1-weighted post-contrast MRI scan shows an intradural extramedullary myxopapillary ependymoma.
This sagittal T1-weighted post-contrast MRI scan shows an intradural extramedullary myxopapillary ependymoma.
Mark H. Bilsky, MD
This sagittal T1-weighted post-contrast MRI scan shows an extradural breast tumor.
This sagittal T1-weighted post-contrast MRI scan shows an extradural breast tumor.
Mark H. Bilsky, MD
This sagittal T2-weighted MRI scan shows a T8-T9 intramedullary ependymoma with syrinx.
This sagittal T2-weighted MRI scan shows a T8-T9 intramedullary ependymoma with syrinx.
Mark H. Bilsky, MD
This sagittal T1-weighted post-contrast MRI scan shows an intradural extramedullary myxopapillary ependymoma.
This sagittal T1-weighted post-contrast MRI scan shows an intradural extramedullary myxopapillary ependymoma.
Mark H. Bilsky, MD
This sagittal T1-weighted post-contrast MRI scan shows an extradural breast tumor.
This sagittal T1-weighted post-contrast MRI scan shows an extradural breast tumor.
Mark H. Bilsky, MD
CT with myelography is an alternative; it can help localize the tumor but provides less information than MRI.
Other spinal masses (eg, abscesses, arteriovenous malformations) and paravertebral tumors can manifest similarly to spinal tumors, but they can typically be distinguished on MRI
Spinal x-rays, taken for other reasons, may show bone destruction, compression fractures, destruction of the vertebral pedicles, or distortion of paraspinal tissues, especially if the tumor is metastatic.
Biopsy may be done to confirm the diagnosis.
Treatment of Spinal Tumors
Glucocorticoids to prevent spinal cord compression
Excision, radiation therapy, or both
If patients have neurologic deficits due to spinal cord compression (eg, myelopathy, cauda equina syndrome), glucocorticoids (eg, dexamethasone, initially intravenous and then oral) are begun immediately to reduce spinal cord edema and preserve function. Tumors compressing the spinal cord are treated as emergencies because deficits may quickly become irreversible.(eg, myelopathy, cauda equina syndrome), glucocorticoids (eg, dexamethasone, initially intravenous and then oral) are begun immediately to reduce spinal cord edema and preserve function. Tumors compressing the spinal cord are treated as emergencies because deficits may quickly become irreversible.
More definitive treatment depends on other factors:
Some well-localized primary spinal tumors can be excised surgically.
Neurologic deficits due to spinal cord compression can be treated with surgery or radiation therapy. Unless tumors are highly radiosensitive (eg, lymphoma, multiple myeloma), surgery is usually done. Deficits may resolve (depending on tumor histology and deficit severity).
Noncompressive metastatic epidural tumors can be treated with conventional external beam radiation or stereotactic radiosurgery, which provide highly durable tumor control.
In the spine-tumor literature, other solid tumor malignancies, such as non-small cell lung and renal cell carcinoma, are differentiated from hormone-driven solid tumor malignancies.
Key Points
Spinal tumors may be intramedullary (within the cord parenchyma) or extramedullary (outside the parenchyma).
Extramedullary tumors may be intradural or extradural.
Most intradural tumors are benign meningiomas, schwannomas, and neurofibromas, which are the most common primary spinal tumors; most extradural tumors are metastatic.
Give glucocorticoids to patients with neurologic deficits due to spinal cord compression.
Surgically excise spinal cord tumors and/or use radiation therapy.
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