Spinal cord tumours (intramedullary and intradural)
Tumours inside or around the spinal cord are rare and usually slow growing, but they press on the cord and threaten walking and bladder control. Most are removed by a surgeon watching nerve signals during the operation; radiotherapy is used when a tumour cannot be fully removed or is high grade, and there are few drugs.
Overview
Spinal cord tumours are grouped by compartment. Intramedullary tumours, inside the cord, are mostly ependymomas in adults (WHO 2021 recognises spinal ependymoma, a MYCN-amplified aggressive subtype, and myxopapillary ependymoma of the filum terminale, now grade 2) and astrocytomas in children, with haemangioblastoma (a third of which are part of von Hippel-Lindau disease) and rare gangliogliomas; diffuse midline gliomas with H3 K27 alteration also occur in the cord and carry that diagnosis. Intradural extramedullary tumours are meningiomas and nerve sheath tumours (schwannoma, neurofibroma), often multiple in NF2-related schwannomatosis and neurofibromatosis type 1. Metastases to the vertebrae and cord compression from them are a separate, far commoner problem handled on the metastatic cancer page.
Surgery with intraoperative neurophysiological monitoring is the mainstay: ependymomas and haemangioblastomas have a plane and can usually be removed completely, which is curative for most; astrocytomas infiltrate and are debulked or biopsied. Radiotherapy follows incomplete resection of ependymoma, any high-grade tumour, and recurrence, with intensity-modulated or proton techniques to spare the cord and adjacent organs. Chemotherapy has a small role, largely temozolomide for high-grade astrocytoma by extrapolation from brain gliomas; belzutifan is approved for von Hippel-Lindau-associated haemangioblastomas that do not need immediate surgery and can shrink cord lesions.
Outcome depends more on neurological state at diagnosis than on any treatment, so early recognition of back pain with progressive weakness or sensory change matters. Long-term follow-up with MRI is standard because recurrence can be late. Molecular classification, including methylation profiling and MYCN status for ependymoma, is beginning to refine prognosis, and NF2-related tumours are the subject of the same targeted trials as vestibular schwannoma.
State of the art
- Intraoperative monitoring has made complete removal of intramedullary ependymoma and haemangioblastoma routine with acceptable neurological risk.
- Belzutifan is the first drug to shrink von Hippel-Lindau haemangioblastomas and defer surgery.
- WHO 2021 gave spinal ependymoma its own type and flagged MYCN amplification as the marker of the aggressive minority.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Check before combiningFood and drink: Temozolomide
Take on an empty stomach or at bedtime to reduce nausea; PJP prophylaxis during concurrent chemoradiation.
See all on the product pages:Temozolomide·Printable cards in the navigator
Anatomy and lymph node drainage
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant gliomaSpinal cord astrocytoma (pilocytic or diffuse, mostly children)
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)Spinal ependymoma (intramedullary, adults; MYCN-amplified subtype aggressive) · Myxopapillary ependymoma of the filum terminale and cauda equina · Spinal cord astrocytoma (pilocytic or diffuse, mostly children) · Diffuse midline glioma, H3 K27-altered, of the spinal cord · Haemangioblastoma (sporadic or von Hippel-Lindau) · Intradural extramedullary meningioma and schwannoma (spinal, often NF2-related)
- Ventricles and ependymal lining (ependymoma)Spinal ependymoma (intramedullary, adults; MYCN-amplified subtype aggressive) · Myxopapillary ependymoma of the filum terminale and cauda equina
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)Intradural extramedullary meningioma and schwannoma (spinal, often NF2-related)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)Intradural extramedullary meningioma and schwannoma (spinal, often NF2-related)
- Pineal and suprasellar midline (germ cell tumours)
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous)
A small fraction of central nervous system tumours, mostly benign or low grade; ependymoma is the commonest intramedullary tumour in adults and astrocytoma in children, and meningioma and schwannoma dominate the intradural extramedullary space.
- DNA methylation profilingEstablished
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Gross total resection with intraoperative neurophysiological monitoring; complete removal is usually curative and needs no adjuvant treatment.
Focal radiotherapy with intensity-modulated or proton techniques; temozolomide for high-grade astrocytoma by extrapolation.
Belzutifan, the HIF-2 alpha inhibitor approved for VHL-associated central nervous system haemangioblastomas, with surveillance imaging.
Resection when symptomatic or growing; radiosurgery for residual or recurrent tumour where the cord dose allows; observation for small asymptomatic lesions.
Subtypes & biomarkers
top- Spinal ependymoma (intramedullary, adults; MYCN-amplified subtype aggressive)
- Myxopapillary ependymoma of the filum terminale and cauda equina
- Spinal cord astrocytoma (pilocytic or diffuse, mostly children)
- Diffuse midline glioma, H3 K27-altered, of the spinal cord
- Haemangioblastoma (sporadic or von Hippel-Lindau)
- Intradural extramedullary meningioma and schwannoma (spinal, often NF2-related)
- Compartment on MRI (intramedullary, intradural extramedullary, extradural)
- MYCN amplification in spinal ependymoma
- H3 K27M status in cord gliomas
- Germline VHL testing for haemangioblastoma
- NF2 germline status in multiple nerve sheath tumours
- Pre-operative neurological grade (McCormick scale)
How often this target appears
- 1887Gowers and Horsley remove a spinal cord tumour
The first successful operation for an intradural spinal tumour.
- 1907Von Eiselsberg resects an intramedullary tumour
- 1993VHL gene identified
Haemangioblastoma of the cord and cerebellum recognised as a hallmark of the syndrome.
- 2021WHO 2021 lists spinal ependymoma, MYCN-amplified, and regrades myxopapillary ependymoma to grade 2
- 2021Belzutifan approved for von Hippel-Lindau tumours including CNS haemangioblastomas
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 7 changes by month →- 2026-09-17This recordSpinal cord tumours (intramedullary and intradural)Facts on this page last checked
When this page itself was last checked or edited.
- 2021GuidelineSpinal cord tumours (intramedullary and intradural)Guideline FDA label 2021: Von Hippel-Lindau haemangioblastoma not needing immediate surgery
Belzutifan, the HIF-2 alpha inhibitor approved for VHL-associated central nervous system haemangioblastomas, with surveillance imaging.
- 2021MilestoneBelzutifanBelzutifan approved for von Hippel-Lindau tumours including CNS haemangioblastomas
A milestone in how this cancer is treated.
- 2021MilestoneSpinal cord tumours (intramedullary and intradural)WHO 2021 lists spinal ependymoma, MYCN-amplified, and regrades myxopapillary ependymoma to grade 2
A milestone in how this cancer is treated.
- 1993MilestoneSpinal cord tumours (intramedullary and intradural)VHL gene identified
Haemangioblastoma of the cord and cerebellum recognised as a hallmark of the syndrome.
- 1907MilestoneSpinal cord tumours (intramedullary and intradural)Von Eiselsberg resects an intramedullary tumour
A milestone in how this cancer is treated.
What is in development for Spinal cord tumours (intramedullary and intradural), drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
Almost no trial evidence; treatment is extrapolated from brain tumours and case series.
Infiltrating cord astrocytomas cannot be removed and have no effective drug.
Radiotherapy to the cord is limited by myelopathy risk.
Neurological deficit at presentation, not treatment, decides most outcomes, so diagnosis is often too late.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
Houston · cancer center | United States | 0 | 6,724 | 95,007 | #2 | ||
Seoul · hospital | South Korea | none recorded | 0 | 1,312 | 17,172 | #3 | |
Rochester, MN · hospital | United States | 0 | 4,511 | 44,748 | #5 | ||
Villejuif · cancer center | France | none recorded | 0 | 1,855 | 31,182 | #6 | |
Seoul · hospital | South Korea | none recorded | 0 | 1,607 | 24,676 | #8 | |
Baltimore · cancer center | United States | 0 | 2,955 | 41,449 | #10 | ||
Berlin · university | Germany | none recorded | 0 | 1,563 | 17,749 | #12 | |
Boston · hospital | United States | 0 | 3,582 | 54,857 | #16 | ||
Heidelberg · cancer center | Germany | none recorded | 0 | 3,456 | 45,745 | #18 | |
Cleveland · hospital | United States | 0 | 2,264 | 29,412 | #20 | ||
Paris · cancer center | France | none recorded | 0 | 1,065 | 15,111 | #21 | |
Seoul · hospital | South Korea | none recorded | 0 | 464 | 3,248 | #22 | |
Manchester · cancer center | United Kingdom | none recorded | 0 | 104 | 2,145 | #23 | |
Stanford · university | United States | 0 | 3,000 | 50,162 | #30 | ||
San Francisco · cancer center | United States | 0 | 2,800 | 46,704 | #33 |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Spinal cord tumours but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Spinal cord tumours
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Compartment on MRI, MYCN amplification in spinal ependymoma, H3 K27M status in cord gliomas, Germline VHL testing for haemangioblastoma, NF2 germline status in multiple nerve sheath tumours), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Spinal ependymoma, Myxopapillary ependymoma of the filum terminale and cauda equina, Spinal cord astrocytoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Intramedullary ependymoma or haemangioblastoma
- For my situation (intramedullary ependymoma or haemangioblastoma), which of the standard options do you recommend and why?Why: Guideline options include: Gross total resection with intraoperative neurophysiological monitoring; complete removal is usually curative and needs no adjuvant treatment.
Incompletely resected, high grade or recurrent
- For my situation (incompletely resected, high grade or recurrent), which of the standard options do you recommend and why?Why: Guideline options include: Focal radiotherapy with intensity-modulated or proton techniques; temozolomide for high-grade astrocytoma by extrapolation.
- Am I a candidate for Temozolomide, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Von Hippel-Lindau haemangioblastoma not needing immediate surgery
- For my situation (von hippel-lindau haemangioblastoma not needing immediate surgery), which of the standard options do you recommend and why?Why: Guideline options include: Belzutifan, the HIF-2 alpha inhibitor approved for VHL-associated central nervous system haemangioblastomas, with surveillance imaging.
- Am I a candidate for Belzutifan, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Intradural extramedullary meningioma or schwannoma
- For my situation (intradural extramedullary meningioma or schwannoma), which of the standard options do you recommend and why?Why: Guideline options include: Resection when symptomatic or growing; radiosurgery for residual or recurrent tumour where the cord dose allows; observation for small asymptomatic lesions.
Any stage
- Are there clinical trials I could join, for example of Belzutifan, Proton therapy, DNA methylation profiling?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Almost no trial evidence; treatment is extrapolated from brain tumours and case series”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Infiltrating cord astrocytomas cannot be removed and have no effective drug”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Spinal cord tumours, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
1drugs
2companies
1terms
1Latest papers
topQuery for this cancer: (TITLE:"Spinal cord tumours" OR ABSTRACT:"Spinal cord tumours" OR TITLE:"intramedullary and intradural" OR ABSTRACT:"intramedullary and intradural" OR TITLE:"Intramedullary spinal cord tumour" OR ABSTRACT:"Intramedullary spinal cord tumour" OR TITLE:"Spinal ependymoma" OR ABSTRACT:"Spinal ependymoma" OR TITLE:"Spinal astrocytoma" OR ABSTRACT:"Spinal astrocytoma" OR TITLE:"Intradural extramedullary tumour" OR ABSTRACT:"Intradural extramedullary tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Spinal cord tumours (intramedullary and intradural), not a curated reading list.
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