Ependymoma
Prepared with OnCo (onco.cc/prep/ependymoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
14 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example Methylation-based molecular group, H3K27me3 loss and EZHIP expression, ZFTA or YAP1 fusion by FISH or sequencing, Chromosome 1q gain and 6q loss, MYCN amplification), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (newly diagnosed intracranial ependymoma, age one year and over), which of the standard options do you recommend and why?
- 6.For my situation (infants under one year or unresectable residual), which of the standard options do you recommend and why?
- 7.Am I a candidate for Vincristine, Carboplatin, Cyclophosphamide or related drugs, and what side effects should I expect?
- 8.For my situation (recurrent), which of the standard options do you recommend and why?
- 9.How do the results of NCI-COG Pediatric MATCH (APEC1621) apply to someone like me?
- 10.Are there clinical trials I could join, for example of DNA methylation profiling, Proton therapy, NCI-COG Pediatric MATCH (APEC1621)?
- 11.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 12.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 13.I read that “PF-A and ZFTA tumours relapse often and have no effective drug; EZHIP/PRC2 and NF-kB dependencies are being tested preclinically and in early trials”. How does that affect my plan?
- 14.I read that “Whether radiotherapy can be omitted or reduced in PF-B, YAP1 and completely resected favourable tumours; SIOP Ependymoma II and COG successors are stratifying by group”. How does that affect my plan?
The words I may hear
- Epigenetic progenitor theory: cancer without a first mutation: The proposal that cancer begins not with a mutation but with a reversible change in how genes are switched on and off in a stem or progenitor cell, which then makes later mutations more likely and more dangerous.
- Late effects and survivorship toxicity: Health problems appearing months or decades after treatment ends: heart damage, infertility, second cancers, lymphoedema, dry mouth, memory problems, weak bones.
Tests and results to bring
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.
Biomarker results to ask for: Methylation-based molecular group, H3K27me3 loss and EZHIP expression (PF-A), ZFTA or YAP1 fusion by FISH or sequencing, Chromosome 1q gain and 6q loss (PF-A risk), MYCN amplification (spinal), Extent of resection on post-operative MRI, Cerebrospinal-fluid cytology and spinal MRI for dissemination.
Scans and tests linked to this cancer: MRI, DNA methylation profiling.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- 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. (Vincristine, Carboplatin, Cyclophosphamide, Etoposide)
- 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. (NCI-COG Pediatric MATCH (APEC1621), Proton therapy)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.