# Spinal cord tumours (intramedullary and intradural)

Source: https://onco.cc/cancers/spinal-cord-tumours/  
OnCo record `spinal-cord-tumours` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Intramedullary spinal cord tumour; Spinal ependymoma; Spinal astrocytoma; Intradural extramedullary tumour; Spinal cord glioma
- Tags: subtype-page; cns
- Group: central nervous system
- Burden: 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.
- Subtypes: 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)
- Biomarkers: 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/spinal-cord-tumours/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/spinal-cord-tumours/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/spinal-cord-tumours/#what-it-is [6 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/spinal-cord-tumours/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/spinal-cord-tumours/#treating-it [4 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/spinal-cord-tumours/#evidence [2 key papers, 5 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/spinal-cord-tumours/#science [1 target]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/spinal-cord-tumours/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/spinal-cord-tumours/#living-with-it [15 questions, 2 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/spinal-cord-tumours/coming/ [2 medicines, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/spinal-cord-tumours/data/ [17 connected records]

## Standard of care

- Intramedullary ependymoma or haemangioblastoma: Gross total resection with intraoperative neurophysiological monitoring; complete removal is usually curative and needs no adjuvant treatment. ([MRI](https://onco.cc/technologies/mri/))
- Incompletely resected, high grade or recurrent: Focal radiotherapy with intensity-modulated or proton techniques; temozolomide for high-grade astrocytoma by extrapolation. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Temozolomide](https://onco.cc/drugs/temozolomide/))
- 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. ([Belzutifan](https://onco.cc/drugs/belzutifan/))
- Intradural extramedullary meningioma or schwannoma: Resection when symptomatic or growing; radiosurgery for residual or recurrent tumour where the cord dose allows; observation for small asymptomatic lesions. ([Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)](https://onco.cc/technologies/radiosurgery-srs/), [Active surveillance](https://onco.cc/technologies/active-surveillance/))

## 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Spinal_tumor
- Wikipedia: https://en.wikipedia.org/wiki/Spinal_tumor
- NCI: adult central nervous system tumours treatment (PDQ): https://www.cancer.gov/types/brain/hp/adult-brain-treatment-pdq

## Connected records

- cancers: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Brain metastases (secondary brain tumours)](https://onco.cc/cancers/secondary-brain-tumours/), [Diffuse midline glioma, H3 K27-altered (including DIPG)](https://onco.cc/cancers/dipg-dmg/), [Ependymoma](https://onco.cc/cancers/ependymoma/), [Meningioma](https://onco.cc/cancers/meningioma/), [Metastatic cancer (cancer that has spread)](https://onco.cc/cancers/metastatic-cancer/), [Vestibular schwannoma (acoustic neuroma)](https://onco.cc/cancers/vestibular-schwannoma/)
- technologies: [Active surveillance](https://onco.cc/technologies/active-surveillance/), [DNA methylation profiling](https://onco.cc/technologies/methylation-profiling/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [MRI](https://onco.cc/technologies/mri/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)](https://onco.cc/technologies/radiosurgery-srs/)
- drugs: [Belzutifan](https://onco.cc/drugs/belzutifan/), [Temozolomide](https://onco.cc/drugs/temozolomide/)
- terms: [Ependymoma molecular groups (PF-A, PF-B, ZFTA, YAP1, MYCN)](https://onco.cc/terms/ependymoma-molecular-groups/), [H3 K27M (diffuse midline glioma)](https://onco.cc/terms/h3k27m/)
- key papers: [Belzutifan for renal cell carcinoma and other tumours in von Hippel-Lindau disease](https://onco.cc/key-papers/paper-belzutifan-vhl-jonasch-nejm-2021/), [The 2021 WHO classification of tumours of the central nervous system: a summary](https://onco.cc/key-papers/paper-who-2021-cns-louis-neuro-oncology-2021/)
- people: [Eric Jonasch](https://onco.cc/people/eric-jonasch/)

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JSON: https://onco.cc/api/v1/entities/spinal-cord-tumours.json