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Ependymoma

A tumor that arises from the ependymal cells lining the ventricles of the brain and the center of the spinal cord.

Location

Brain and spinal cord

Histological Classification

Ependymal Tumor


Details

Origin: Ependymal cells

Grade: II-III

Severity: Moderate to High

Molecular Marker: RELAFusion, C11orf95

Age Range: 1-5 years

Life Expectancy: 5-15 years

Lifespan Impact

Summary

Ependymomas are a type of tumor that arises from ependymal cells, which line the ventricles of the brain and the central canal of the spinal cord. They belong to the broader category of ependymal tumors. These tumors can occur in both children and adults, but they are more common in children and are often located in the brain, whereas in adults, they may occur more frequently in the spinal cord.

Symptoms

The symptoms of ependymoma can vary depending on the tumor's location. Common symptoms include:

  • Intracranial Ependymomas (Brain)

    • Headaches
    • Nausea and vomiting
    • Balance and coordination difficulties
    • Changes in vision or eye movement
    • Seizures
    • Behavioral changes
  • Spinal Ependymomas

    • Back pain
    • Weakness or numbness in the arms or legs
    • Difficulty walking
    • Loss of bladder or bowel control

Treatments and Cures

The primary goal of ependymoma treatment is to remove as much of the tumor as possible while preserving neurological function. Treatment options include:

  1. Surgery

    • Modern Techniques: The primary treatment for ependymomas is surgical resection. Advances in surgical techniques, including microsurgery and intraoperative MRI, have improved the ability to remove these tumors safely.
  2. Radiation Therapy

    • Post-surgical radiation is commonly used to target any remaining tumor cells. Techniques such as conformal radiation therapy and intensity-modulated radiation therapy (IMRT) help minimize damage to surrounding healthy tissue.
  3. Chemotherapy

    • While chemotherapy is not the primary treatment for ependymomas, it may be used in certain cases, particularly when the tumor cannot be completely resected or in recurrent cases. Drugs such as temozolomide and platinum-based agents might be utilized.

Experimental Treatments

Research into new treatments for ependymomas is ongoing, with several experimental therapies under investigation:

  1. Targeted Therapy

    • Molecular profiling of tumors may identify specific mutations or pathways that can be targeted with drugs. Clinical trials are exploring agents that target these pathways.
  2. Immunotherapy

    • Immunotherapy aims to harness the body's immune system to recognize and destroy tumor cells. Trials are underway to evaluate the efficacy of various immunotherapeutic approaches for ependymomas.
  3. Proton Beam Therapy

    • This advanced form of radiation therapy uses protons instead of traditional X-rays, allowing for more precise targeting of tumor tissue with less impact on surrounding healthy tissue.

Side Effects of Treatments

Each treatment option comes with potential side effects:

  • Surgery

    • Risks include infection, bleeding, and potential neurological deficits depending on the tumor's location.
  • Radiation Therapy

    • May cause fatigue, skin changes, and in some cases, cognitive effects or hormonal imbalances, especially in children.
  • Chemotherapy

    • Common side effects include nausea, fatigue, hair loss, and increased risk of infection.
  • Experimental Treatments

    • Side effects can vary widely and are often specific to the type of treatment being administered. These are closely monitored in clinical trials.

Timeline of Modern Treatments

  • Surgical Advances: Techniques such as microsurgery have been evolving since the late 20th century, with intraoperative imaging becoming more prevalent in the 2000s.
  • Radiation Therapy: IMRT and conformal techniques have been in use since the early 2000s, with proton therapy becoming more accessible in the 2010s.
  • Chemotherapy: Although not the primary treatment, chemotherapy agents have been used for several decades, with ongoing research into more effective combinations.
  • Experimental Therapies: Targeted therapies and immunotherapies are at the forefront of current research, with many trials initiated in the 2010s and continuing today.

Continued research and clinical trials aim to improve outcomes for patients with ependymoma, offering hope for more effective and less harmful treatment options in the future.