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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Newly diagnosed intracranial ependymoma, age one year and over | 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. | not mapped |
| Infants under one year or unresectable residual | Chemotherapy (vincristine, carboplatin, cyclophosphamide, etoposide-based) to delay radiotherapy or facilitate second surgery, per SIOP Ependymoma II and COG protocols. | not mapped |
| Recurrent | 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. | not mapped |