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Ependymomas grow from the cells lining the fluid spaces of the brain and spinal cord, mostly in children under five. Removing the whole tumour followed by focused radiotherapy controls most cases; molecular groups defined in 2021 behave differently, with posterior fossa group A relapsing often, and there is no approved drug.
Ependymoma is a glial tumour arising along the ventricular system and spinal canal. The WHO 2021 classification replaced grade-based labels with molecular groups that carry prognostic weight: posterior fossa group A (PF-A, infants and young children, CpG hypermethylation with EZHIP overexpression and loss of H3K27me3, chromosome 1q gain marks high risk), posterior fossa group B (PF-B, older children and adults, favourable), supratentorial ZFTA (formerly RELA) fusion-positive (NF-kB driven), supratentorial YAP1 fusion-positive (young children, favourable), and spinal groups including myxopapillary ependymoma and MYCN-amplified spinal ependymoma. Few recurrent point mutations exist; the disease is largely driven by structural and epigenetic changes.
Treatment is surgical: gross total resection is the strongest modifiable prognostic factor, and second-look surgery for residual disease is standard practice. Post-operative conformal radiotherapy to the tumour bed, including in children as young as one year, was established by the St Jude RT1 and COG ACNS0121 studies and controls disease without the whole-brain exposure of earlier eras; proton therapy is increasingly used to spare cochlea, hypothalamus and healthy brain. Chemotherapy has a limited role: it is used to bridge infants to radiotherapy or to enable second surgery, and the COG trial ACNS0831 tested maintenance chemotherapy after radiotherapy without establishing it as a universal standard. There is no approved targeted drug.
The active fronts are molecular risk stratification (SIOP Ependymoma II, ACNS0831 successors), reducing radiotherapy in favourable groups such as PF-B and ST-YAP1, and finding therapies for PF-A and ZFTA tumours, where relapse is common and repeated surgery and re-irradiation are the mainstay. Epigenetic dependencies (EZHIP and PRC2 biology in PF-A, NF-kB signalling in ZFTA), CDK4/6 inhibition and immunotherapy against fusion neoantigens are in preclinical and early clinical work.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Roughly one in ten childhood brain tumours, most under five years of age; also occurs in adults, mainly in the spine (NCI PDQ).
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.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), 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), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Maximal safe resection, second-look surgery for residual disease, then conformal or proton radiotherapy to the tumour bed (ACNS0121 approach); craniospinal irradiation only for disseminated disease.
Chemotherapy (vincristine, carboplatin, cyclophosphamide, etoposide-based) to delay radiotherapy or facilitate second surgery, per SIOP Ependymoma II and COG protocols.
Repeat resection and re-irradiation (focal or craniospinal) where feasible; no standard systemic therapy, so trial enrolment (including Pediatric MATCH-style molecular assignment) is recommended.
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Maximal resection followed by conformal radiotherapy is the mainstay for most childhood ependymoma, including in very young children; incompletely resected tumours need better approaches.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Ependymoma" OR ABSTRACT:"Ependymoma" OR TITLE:"Childhood ependymoma" OR ABSTRACT:"Childhood ependymoma" OR TITLE:"Posterior fossa ependymoma" OR ABSTRACT:"Posterior fossa ependymoma" OR TITLE:"Supratentorial ZFTA fusion ependymoma" OR ABSTRACT:"Supratentorial ZFTA fusion ependymoma" OR TITLE:"Myxopapillary ependymoma" OR ABSTRACT:"Myxopapillary ependymoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Ependymoma, not a curated reading list.
St Jude RT1 (Merchant, Lancet Oncology) shows tumour-bed irradiation controls disease with preserved cognition.
Witt and colleagues (Cancer Cell) separate PF-A and PF-B by expression.
Parker and colleagues (Nature) link the fusion to NF-kB activation.
Pajtler and colleagues (Cancer Cell) define the methylation-based classification later adopted by WHO.
COG phase 2 (JCO 2019) reports outcomes by extent of resection and molecular group.
The targets of this cancer's medicines and the ones linked to it directly.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Fatal if given intrathecally: label all syringes.
Dose by Calvert formula using GFR (see the calculators).
Reduce to 75% for CrCl 15-50.
The leukaemia risk after chemotherapy was described in the era of mustards and etoposide, and it did not stay there. Platinum drugs carry it, PARP inhibitors raise it about two and a half times against placebo, and lenalidomide with oral melphalan raises it nearly fivefold against melphalan alone. The absolute numbers are small, but the choice of partner drug is sometimes a real decision.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
See all on the product pages:CarboplatinCyclophosphamideEtoposideVincristine·Printable cards in the navigator
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