Your Visual Guide Spinal Cord Tumors

Understand this diagnosis through data, infographics, and evidence based on modern classification and current research.

~5,000
New Spinal Cord Tumor Cases/Year (U.S.)
Understanding Spinal Cord Tumors
What Are Spinal Cord Tumors & How Are They Classified?
Spinal cord tumors are classified by anatomic compartment: intramedullary (within the cord), intradural-extramedullary (inside the dura but outside the cord), and extradural. Each has distinct tumor types and prognosis.
Spinal Cord Tumor
Anatomic Compartment?
Intramedullary
Intramedullary (~20%)
Within the spinal cord
Astrocytoma Ependymoma Hemangioblastoma
Intradural-Extramedullary
Intradural-Extramedullary (~40%)
Inside dura, outside cord
Schwannoma Spinal Cord Tumors Neurofibroma
Extradural
Extradural (~40%)
Outside the dura
Metastases Chordoma Chondrosarcoma
Tumor Grading
Spinal Cord Tumor Classification
Spinal cord tumors are classified by anatomic compartment (intramedullary, intradural-extramedullary, or extradural) and histology.
1
Intramedullary Astrocytoma
Most common intramedullary tumor in children. Low-grade (pilocytic, diffuse) and high-grade variants. Infiltrative growth limits resection.
2
Intramedullary Ependymoma
Most common intramedullary tumor in adults. Well-circumscribed, amenable to GTR. Myxopapillary variant at conus/filum.
3
Hemangioblastoma
Highly vascular intramedullary tumor. ~25% associated with VHL syndrome. Curable with surgery when complete resection achieved.
4
Intradural-Extramedullary
Spinal Cord Tumors and schwannomas most common. Well-circumscribed, surgically curable. Spinal Cord Tumors predominantly in women.
5
Chordoma
Arises from notochordal remnants. Sacral most common, also clival and mobile spine. Brachyury-positive. Proton therapy important.
6
Extradural Tumors
Most are metastatic. Primary vertebral tumors rare (chondrosarcoma, osteosarcoma, Ewing sarcoma). Epidural compression is emergency.
Molecular Markers
The Key Biomarkers
Modern classification depends on specific molecular markers. Each reveals something different about the tumor.
Diagnostic
H3K27M
Histone H3 K27M Mutation
Defines diffuse midline glioma including spinal cord. Poor prognosis. WHO Grade 4 regardless of histology.
Diffuse midline
Diagnostic
IDH1/2
Isocitrate Dehydrogenase
Mutations found in some spinal astrocytomas. IDH-mutant tumors have better prognosis than IDH-wildtype.
Astrocytoma
Diagnostic
Brachyury
T-Box Transcription Factor
Nuclear expression is pathognomonic for chordoma. Essential diagnostic marker. Potential therapeutic target.
Chordoma defining
Predictive
VHL
Von Hippel-Lindau
Germline mutations cause VHL syndrome: hemangioblastomas, clear cell RCC, pheochromocytoma. Lifelong surveillance.
Hemangioblastoma
Prognostic
CDKN2A/B
Cyclin-Dependent Kinase Inhibitor
Homozygous deletion in spinal ependymoma associated with higher grade and worse prognosis.
Ependymoma prognostic
Diagnostic
EMA
Epithelial Membrane Antigen
Dot-like perinuclear staining pattern in ependymoma. Helps distinguish from astrocytoma.
Ependymoma marker
Signs & Symptoms
Spinal Cord Tumor Signs & Symptoms
Symptoms depend on tumor location (cervical, thoracic, lumbar) and compartment (intra- vs extramedullary).

Pain

Localized back/neck pain, often worse at night or with recumbency. Radicular pain following nerve root distribution.

Motor Weakness

Progressive weakness below tumor level. May be asymmetric. Upper motor neuron signs (spasticity, hyperreflexia).

Sensory Changes

Numbness, paresthesias, proprioceptive loss. Cape-like sensory loss in intramedullary tumors (central cord pattern).

Bowel/Bladder Dysfunction

Late symptom indicating significant cord compression. Urinary retention or incontinence, constipation.

Gait Abnormality

Ataxic or spastic gait depending on tumor location. Progressive difficulty walking is common presentation.

Brown-Sequard Syndrome

Ipsilateral motor weakness and proprioceptive loss with contralateral pain/temperature loss. Classic but rare.

Diagnosis
The Diagnostic Journey
From first MRI to surgical planning — the path to a spinal cord tumor diagnosis.
1

MRI Spine

Gadolinium-enhanced MRI of the entire spine is the gold standard for evaluation

2

MRI Brain

To evaluate for intracranial drop metastases or associated CNS lesions

3

CT Myelography

Useful when MRI is contraindicated or for surgical planning of bony involvement

4

Neurophysiology

EMG/NCS to characterize nerve root involvement and baseline function

5

Biopsy/Surgery

Intraoperative neurophysiological monitoring (MEP/SSEP) guides safe resection

6

Molecular Testing

H3K27M, IDH status, brachyury for diagnosis and prognosis

Treatment
Treatment Options for Spinal Cord Tumors
Treatment balances tumor control with preservation of neurological function. Intraoperative monitoring is essential.

Microsurgery

Primary treatment for most spinal cord tumors. GTR when safely achievable. Intraoperative neurophysiology monitoring (MEP/SSEP) is mandatory.

Radiation Therapy

Adjuvant for incompletely resected tumors. Proton therapy preferred for chordoma. IMRT for spinal ependymoma/astrocytoma.

Observation

For small, asymptomatic, or stable tumors. Serial MRI monitoring. Appropriate for some low-grade tumors with stable neurology.

Stereotactic Radiosurgery

CyberKnife for small, well-defined spinal tumors. Single or hypofractionated treatment. Limited by cord tolerance.

Chemotherapy

Limited role. Temozolomide for high-grade astrocytoma/H3K27M. Imatinib investigated for chordoma.

Clinical Trials

Targeted therapies, immunotherapy, and novel radiation approaches under investigation.

Find matching trials →
Care Team
Your Multidisciplinary Care Team
Spinal cord tumor surgery requires a highly specialized team with intraoperative neurophysiological monitoring expertise.
Core Specialists

Neurosurgeon

Primary specialist for surgical resection and skull base approaches

Radiation Oncologist

Plans and delivers radiation therapy and stereotactic radiosurgery

Neuro-oncologist

For higher-grade or refractory spinal cord tumors requiring systemic therapy

Support Team

Neuroradiologist

Specialized imaging interpretation and monitoring

Neuropathologist

Tissue analysis, WHO grading, and molecular profiling

Nurse Navigator

Guides you through appointments, insurance, and logistics

Resources
Support Organizations
Organizations providing information, community, and support for spinal cord tumor patients.

Chordoma Foundation

Dedicated to accelerating the development of treatments and ultimately a cure for chordoma.

Visit Chordoma Foundation →

VHL Alliance

Supporting von Hippel-Lindau syndrome patients with hemangioblastoma and other manifestations.

Visit VHL Alliance →

National Brain Tumor Society

Leading nonprofit investing in research, advocacy, and patient services for all brain tumors.

Visit National Brain Tumor Society →

SpinalCord.com

Resources and community support for patients with spinal cord conditions including tumors.

Visit SpinalCord.com →

Want to Explore Clinical Trials?

Search clinical trials for spinal cord tumors and find the ones that match your specific diagnosis.

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