Spinal Ependymoma
Most common primary intramedullary spinal cord tumor in adults
ICD-10: D33.4 · tumors condition
Spinal ependymomas are the most common primary intramedullary (within the cord substance) spinal cord tumors in adults, accounting for approximately 60% of intramedullary spinal cord tumors. They arise from ependymal cells lining the central canal of the spinal cord or the filum terminale. The cervical cord is the most common site for cellular ependymomas; myxopapillary ependymomas arise specifically from the conus medullaris and filum terminale and are considered a separate, generally lower-grade variant. Spinal ependymomas typically grow slowly and are often well-demarcated from surrounding cord tissue, creating a favorable surgical plane. This is in contrast to astrocytomas, the other major intramedullary tumor, which are typically infiltrative and more difficult to resect completely. Presenting symptoms evolve over months to years: posterior column dysfunction (sensory ataxia, loss of vibration), motor weakness, and bladder dysfunction. A cap sign (hemosiderin staining at the tumor poles) on MRI is characteristic. Gross total resection is the goal and is achievable in most cellular ependymomas. Ten-year recurrence-free survival after gross total resection exceeds 85%. Subtotal resection followed by adjuvant radiotherapy is used when gross total resection risks unacceptable cord injury. Myxopapillary ependymomas of the filum terminale have a higher local recurrence rate and may require adjuvant radiation after resection.
Anatomy & Pathology
The ependymal cell layer lines the central canal that runs the length of the spinal cord. At the level of the conus medullaris, the canal opens into the filum terminale, a thin strand of glial and fibrous tissue anchoring the cord. Myxopapillary ependymomas arise here. Cellular ependymomas arise from remnant ependymal cells scattered throughout the cord parenchyma, most commonly in the cervical region.
Symptoms
- Back or neck pain — often the first symptom, sometimes for years before diagnosis
- Sensory ataxia and loss of vibration and proprioception (posterior column involvement)
- Bilateral leg weakness and spasticity progressing over months to years
- Bladder dysfunction: urgency, hesitancy, retention
- Radicular or band-like pain at the level of the tumor
- Scoliosis from cord dysfunction (common in pediatric ependymoma)
- Cap sign on MRI (hemosiderin poles) suggesting ependymoma over astrocytoma
Causes & Risk Factors
- NF2 gene mutation: neurofibromatosis type 2 associated with multiple spinal ependymomas
- Sporadic mutation in ependymal cells lining the central canal (most cases)
- Prior spinal irradiation as a rare precipitating factor
- MYCN amplification in aggressive (Grade 3) ependymomas
Imaging Findings
Imaging studies are commonly used to identify findings associated with this condition. Results vary by individual; a qualified spine specialist interprets findings in the context of a full clinical evaluation.
MRI
- Intramedullary mass that is isointense to the cord on T1 and hyperintense on T2, often centrally located within the cord
- Cap sign: hemosiderin deposits at the superior and inferior poles of the tumor appearing as T2 hypointense caps — highly characteristic of ependymoma
- Vivid, homogeneous gadolinium enhancement in cellular ependymoma
- Syringomyelia rostral or caudal to the tumor is common — associated cord cavity from CSF flow disruption
- Myxopapillary ependymoma: well-defined intradural extramedullary mass at the conus or filum terminale, often with heterogeneous enhancement
CT Scan
- Spinal canal widening or scalloping of the posterior vertebral bodies from slow-growing tumor expansion
- Rarely helpful for tumor characterization — MRI with gadolinium is required
- CT myelogram in patients with MRI contraindication can demonstrate the intramedullary filling defect
X-Ray
- Posterior vertebral body scalloping or widened interpedicular distance in large or longstanding tumors
- Scoliosis may develop as a consequence of the intramedullary lesion — seen on full-length scoliogram
- Plain films are not adequate for diagnosis; MRI is required for characterization
Who Is Commonly Affected
The following patterns are commonly associated with this condition based on published population studies. Individual presentation varies; these figures are informational only.
Peak Age Range
40–60 years for intramedullary ependymoma; myxopapillary ependymoma has a wider age range (20–50 years)
Gender Distribution
Slight male predominance for intramedullary ependymoma; roughly equal for myxopapillary type
Estimated Prevalence
Ependymomas account for approximately 60–70% of all intramedullary spinal cord tumors in adults; myxopapillary ependymoma is the most common tumor of the cauda equina and filum terminale; annual incidence approximately 1–2 per million
Treatment Options
Conservative
- MRI surveillance every 6–12 months for incidentally found, asymptomatic, small lesions in high-surgical-risk patients
- Corticosteroids for perioperative cord edema management
- Rehabilitation for neurological deficits: physical therapy, bladder management
Surgical
- Microsurgical gross total resection via laminectomy — goal of surgery; achieves cure in most Grade 1–2 ependymomas
- Subtotal resection when gross total resection risks cord injury, followed by adjuvant radiotherapy
- Stereotactic radiosurgery (SRS) for recurrent or residual lesion not amenable to re-resection
When to see a spine specialist
Progressive myelopathy — sensory changes, leg weakness, or bladder dysfunction — without a structural compressive explanation on standard imaging should prompt gadolinium-enhanced MRI to assess for intramedullary lesion. Diagnosis is often delayed; patients are frequently managed for other spine conditions for months to years before ependymoma is recognized.
Specialists Who Treat Spinal Ependymoma
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Questions to Ask Your Doctor
Bring these questions to your next appointment about spinal ependymoma.
- 1
What is the location and WHO grade of my ependymoma — is this a myxopapillary ependymoma (filum terminale), cellular ependymoma (intramedullary), or another subtype?
- 2
Is complete surgical resection achievable for my tumor, and what is the likelihood of cure with gross total resection versus the risk of neurological deficit?
- 3
Is post-operative radiation therapy recommended for my grade or resection completeness, and what are the long-term risks of spinal radiation?
- 4
What is the recommended surveillance schedule — and how long must I be followed before I can be considered in long-term remission?
- 5
Are there any hereditary syndromes (NF2, neurofibromatosis type 2) I should be screened for given this diagnosis?
Research Evidence
No studies reviewed yet for this condition. Check back soon — our evidence pipeline runs nightly.
Clinical Evidence
Frequently Asked Questions
How is a spinal ependymoma different from a spinal astrocytoma?
Both are intramedullary tumors, but ependymomas typically have a well-defined pseudocapsule and a surgical plane from surrounding cord tissue, enabling gross total resection in most cases. Astrocytomas are usually infiltrative without a clear margin, making total resection difficult and risky. Ependymomas account for 60% of adult intramedullary tumors; astrocytomas predominate in children. MRI characteristics also differ: ependymomas often show a characteristic cap sign and are centrally located, while astrocytomas are more eccentric.
What is a myxopapillary ependymoma?
Myxopapillary ependymoma (MPE) is a distinct WHO Grade 1 variant that arises almost exclusively from the conus medullaris and filum terminale. It is the most common tumor of the cauda equina region and is characterized by a mucinous matrix and papillary architecture. MPEs grow slowly and present with low back pain and radicular symptoms over years. Despite being benign, MPEs have a higher local recurrence rate after subtotal resection and may seed the CSF. Gross total resection with intact pseudocapsule gives the best long-term outcomes.
Do I need radiation after surgery for a spinal ependymoma?
For WHO Grade 1–2 spinal ependymomas with confirmed gross total resection, adjuvant radiation is generally not recommended — surgery alone provides excellent 10-year recurrence-free survival. Radiation is reserved for: subtotal resection where re-operation is not feasible, Grade 3 (anaplastic) ependymomas, and recurrent tumors. For myxopapillary ependymomas of the filum terminale, the role of adjuvant radiation after subtotal resection is debated; some centers recommend it given the higher local recurrence rate.