What Is Ependymoma? Definition: Ependymoma is a rare tumor arising from ependymal cells, the cells that line the ventricles (fluid-filled spaces) of the brain and the central canal of the spinal cord. (Johns Hopkins Medicine) Location: Can occur in the brain (intracranial) or spinal cord. (Cancer.gov) Nature: It’s considered a primary central nervous system (CNS) tumor — meaning it originates in the CNS, not from another part of the body. (Johns Hopkins Medicine)

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Epidemiology & Risk Factors

Types & Grading

According to pathology and molecular classification:

Molecular subtypes (per WHO / NCI):

  • Supratentorial (in the brain) — ZFTA fusion-positive, YAP1 fusion-positive (Cancer.gov)

  • Posterior fossa (at the base of brain) — Group A (PFA), Group B (PFB) (Cancer.gov)

  • Spinal: spinal ependymoma, and a rare MYCN-amplified variant (Cancer.gov)

How It Causes Symptoms

  • Tumor mass effect: as it grows, it can press on brain or spinal structures, leading to neurological symptoms. (Johns Hopkins Medicine)

  • CSF flow obstruction: Especially in intracranial tumors, they can block normal cerebrospinal fluid (CSF) flow → hydrocephalus, raising intracranial pressure. (braintumorcenter.ucsf.edu)

  • Spread within CNS: Ependymoma cells can disseminate through CSF to other parts of the brain or spinal cord. (Cancer.gov)

Symptoms

Depends heavily on location:

Diagnosis

To confirm and classify ependymoma:

  1. Neurological exam: Check strength, coordination, reflexes, vision, balance. (Mayo Clinic)

  2. Imaging:

    • MRI is the main tool (brain + spinal MRI, depending on suspicion) (Mayo Clinic)

    • With contrast to highlight the tumor. (Cancer.gov)

  3. Surgical biopsy / resection: Tissue is removed (if possible) and examined under a microscope by a neuropathologist. (Cancer.gov)

  4. CSF analysis: Sometimes a lumbar puncture (spinal tap) is done to check for tumor cells in the cerebrospinal fluid. (Mayo Clinic)

  5. Molecular analysis: Increasingly, tumors are classified by genetic/molecular markers (e.g. ZFTA fusion, MYCN amplification) for prognosis and treatment planning. (American Brain Tumor Association)

Treatment

Depends on grade, location, how much tumor can be removed, molecular subtype, and patient factors. Main strategies:

  1. Surgery

    • Aim: remove as much as safely possible (“maximal safe resection”). (Mayo Clinic)

    • If complete removal is achieved and there are low-risk features, further therapy may or may not be needed. (Mayo Clinic)

  2. Radiation therapy

    • Used after surgery, especially if resection was incomplete or for higher-grade tumors. (Mayo Clinic)

    • Techniques: conformal radiation, intensity-modulated RT, or proton therapy (in specialized centers) to minimize damage to healthy tissue. (Mayo Clinic)

    • Stereotactic radiosurgery: a focused high-dose radiation option in selected cases. (Mayo Clinic)

  3. Chemotherapy

    • Less commonly used than in some other brain tumors. (Mayo Clinic)

    • May be considered when tumor recurs, or in certain high-risk settings. (Cleveland Clinic)

  4. Targeted therapy / Clinical trials

    • Because of the molecular heterogeneity of ependymomas, targeted treatments (against specific fusions or amplifications) are being studied. (Mayo Clinic)

    • Clinical trials are often recommended, especially for recurrent or high-grade disease. (Cancer.gov)

Prognosis

  • Five-year survival: According to the NCI, about 88.2% overall, but this varies a lot depending on grade, location, molecular subtype, extent of resection, and age. (Cancer.gov)

  • Recurrence: Even after treatment, ependymomas (especially higher grade) can recur. (Johns Hopkins Medicine)

  • Prognostic factors:

Challenges & Research Directions

  • Molecular classification: The newer WHO classification (2021) emphasizes genetic/molecular features over just histology. (Cancer.gov)

  • Radiation toxicity: Because these tumors are in sensitive CNS regions, balancing effective radiation with minimizing harm is a major challenge.

  • Novel radiotherapy techniques: Research into proton therapy, and more recently intensity-modulated proton arc therapy (IMPAT), aims to reduce dose to healthy brain/spine tissue. (arXiv)

  • Intraoperative imaging: New technologies like robotic optical coherence tomography (OCT) are being explored to better guide spinal cord tumor surgery. (arXiv)

  • Segmentation & AI: Tools like deep learning for MRI segmentation of spinal cord tumors (including ependymoma) are being developed for better diagnosis and surgical planning. (arXiv)

Living With Ependymoma / Follow-Up

  • Regular MRI scans: After treatment, periodic MRI scans are necessary to monitor for recurrence. (Johns Hopkins Medicine)

  • Neurological follow-up: Because the tumor (or its treatment) can affect neurological function, ongoing care by neurologists/neuro-oncologists is important.

  • Supportive care: Includes physical therapy, pain management, neuro-rehabilitation, and possibly psychosocial support.

  • Clinical trials: Many patients are encouraged to consider clinical trials, especially for recurrent disease or for molecularly defined subtypes.

Summary / Key Take-Home Points

  • Ependymoma is a rare CNS tumor from ependymal cells, in brain or spinal cord.

  • Causes are mostly unknown, though NF2 is a risk factor.

  • Grading (I–III) and molecular subtype are critical for prognosis.

  • Surgery is the mainstay; radiation is often used; chemotherapy less so; clinical trials are very important.

  • Prognosis is relatively favorable in many cases, but recurrence is a real risk.

  • Research is active in new imaging, radiation, and molecular therapies.

If you like, I can check recent (last 5 years) research progress on ependymoma (clinical trials, therapies) — do you want me to do that?

 


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