Anaplastic ependymoma cancer: Symptoms, treatment, and more

Introduction

Anaplastic ependymoma is a rare and aggressive form of cancer that originates in the brain or spinal cord. While ependymomas can affect people of all ages, anaplastic variants tend to grow rapidly and exhibit irregular cellular structures, making them particularly challenging to treat. This form of cancer is categorized as a grade 3 tumor, indicating its malignant and fast-growing nature.

Understanding the characteristics, symptoms, treatment options, and prognosis of anaplastic ependymoma is vital for those diagnosed with the condition—as well as their families and caregivers. This comprehensive guide will walk you through everything you need to know about this rare disease, including diagnosis methods, therapeutic strategies, and coping mechanisms.

---

What Is Anaplastic Ependymoma?

Anaplastic ependymoma is a type of cancerous tumor that develops in the cells lining the ventricular system of the brain and the central canal of the spinal cord. These cells are responsible for producing cerebrospinal fluid (CSF), which cushions and protects the brain and spinal cord.

Ependymomas are categorized into three grades:

• Grade 1: Benign and slow-growing
• Grade 2: Low-grade tumors with moderate growth
• Grade 3 (Anaplastic): Malignant, fast-growing tumors with abnormal cell structures

Anaplastic ependymomas are classified as grade 3 because the cancer cells are unusually shaped, divide rapidly, and tend to invade surrounding tissues more aggressively than their lower-grade counterparts.

These tumors may occur in both the brain and the spinal cord:

• Children are more likely to develop brain-based ependymomas.
• Adults tend to develop these tumors in the spinal cord.

Due to their rarity—comprising only about 1.7% of all brain tumors—anaplastic ependymomas often require specialized diagnosis and care.

---

Symptoms of Anaplastic Ependymoma

The symptoms of anaplastic ependymoma largely depend on the tumor’s location within the central nervous system. These tumors can block the flow of cerebrospinal fluid or press against nearby neural structures, leading to a wide range of symptoms.

Brain-Based Symptoms

Tumors in the brain typically result in increased intracranial pressure and neurological symptoms, such as:

• Persistent headaches
• Nausea and vomiting
• Dizziness and balance issues
• Vision problems (blurry or double vision)
• Seizures
• Changes in mood or behavior

In infants and young children, symptoms may include an enlarged head size or a bulging soft spot on the skull (fontanel).

Spinal Cord Symptoms

If the tumor develops in the spinal cord, it may affect sensory and motor functions:

• Chronic back pain
• Numbness or tingling in the limbs
• Muscle weakness in arms, legs, or torso
• Difficulty walking or maintaining balance
• Urinary or bowel incontinence
• Sexual dysfunction

Because symptoms can mirror other neurological conditions, early imaging and accurate diagnosis are essential to avoid delays in treatment.

---

What Causes Anaplastic Ependymoma?

Like many cancers, anaplastic ependymoma develops when normal cells undergo genetic mutations that disrupt their regular growth cycles. These mutations cause cells to grow uncontrollably and form tumors. Several potential causes and risk factors include:

Genetic Factors

• Spontaneous mutations: DNA changes during cell replication without any external cause.
• Inherited mutations: Less common, but possible in cases with a family history of CNS tumors.

Environmental Triggers

While no definitive environmental cause has been established for anaplastic ependymoma, general cancer risk factors may apply:

• Exposure to radiation
• Carcinogens like tobacco smoke
• Prolonged exposure to industrial chemicals
• Possibly UV light (indirectly related)

Unfortunately, the exact cause remains largely unknown, and more research is needed to pinpoint specific triggers.

---

How Is Anaplastic Ependymoma Diagnosed?

Diagnosing anaplastic ependymoma involves a combination of neuroimaging, physical exams, and pathology.

MRI Scans

• Magnetic Resonance Imaging (MRI) is the gold standard for detecting tumors within the brain and spinal cord.
• These scans help determine the size, location, and potential grade of the tumor.

Biopsy

• A neurosurgeon may perform a surgical biopsy to extract a sample of the tumor.
• This tissue is examined by a neuropathologist, who confirms the diagnosis and assesses how aggressive the cancer is.

In some cases, a lumbar puncture (spinal tap) may also be performed to test cerebrospinal fluid for cancer cells, especially if metastasis is suspected.

Early and accurate diagnosis is crucial for choosing the most effective treatment strategy and improving the chances of long-term survival.

---

Treatment Options for Anaplastic Ependymoma

Due to its aggressive nature, treating anaplastic ependymoma often requires a multifaceted approach. The treatment plan typically depends on several factors:

• Tumor location and size
• The patient’s age
• Whether the tumor has spread
• Response to prior treatments (if recurrent)

1. Surgery

• Primary method for removing the tumor.
• The goal is complete resection, but this is not always possible due to tumor location near vital structures.
• The amount of tumor removed can significantly affect prognosis.

2. Radiation Therapy

• Recommended post-surgery to destroy any remaining cancer cells.
• Focal radiation is often used to minimize damage to healthy brain or spinal tissue.
• Proton therapy may also be considered in pediatric cases to reduce long-term side effects.

3. Chemotherapy

• Less effective as a first-line treatment for ependymoma but may be used in certain cases.
• Sometimes used when surgery or radiation is not possible or as part of a clinical trial.

4. Rehabilitative Therapies

• Especially important for patients recovering from surgery.
• May include physical therapy, occupational therapy, and speech therapy, depending on the tumor’s impact.

Because ependymomas are rare, many patients benefit from seeking care at a cancer center with experience treating central nervous system tumors.

Outlook and Prognosis for Anaplastic Ependymoma

Prognosis for anaplastic ependymoma varies widely and depends on a combination of biological, clinical, and treatment-related factors. While this form of ependymoma is considered aggressive, advances in surgical techniques and targeted therapies have significantly improved outcomes for many patients.

Understanding the 5-Year Survival Rate

The 5-year survival rate for all types of ependymoma is approximately 83.9%, meaning that about 84 out of 100 individuals diagnosed are still alive five years after diagnosis. However, it’s important to note that this rate encompasses all grades and types of ependymoma—not just the anaplastic form.

For grade 3 anaplastic ependymomas, the outlook is generally less favorable due to the tumor’s rapid growth and tendency to recur. Still, some patients experience long-term survival, particularly if the tumor is fully removed and responsive to radiation therapy.

Factors That Influence Prognosis

• Tumor Grade: Grade 3 tumors are more aggressive than grades 1 or 2.
• Tumor Location: Tumors in accessible areas are easier to remove surgically.
• Extent of Resection: Patients who undergo complete tumor removal tend to have better outcomes.
• Age: Younger children, especially under 3 years of age, often have a poorer prognosis, while older children and adults may fare better.
• Recurrence: Recurrent tumors are harder to treat and often require additional surgery or experimental therapies.
• Response to Treatment: Some tumors are more resistant to radiation or chemotherapy, affecting the likelihood of long-term remission.

It’s also worth mentioning that individual response to therapy plays a significant role. Two people with similar diagnoses can have very different experiences based on their overall health, genetic profile, and tumor biology.

---

Potential Complications of Anaplastic Ependymoma and Its Treatment

Both the disease itself and its treatment carry potential complications, some of which may be long-term or even permanent. These complications vary based on tumor location and the type of intervention used.

1. Hydrocephalus

When a tumor blocks the normal flow of cerebrospinal fluid, it can lead to a dangerous buildup known as hydrocephalus. This condition causes the ventricles in the brain to swell and increases pressure inside the skull.

Symptoms in Infants

• Enlarged head
• Bulging fontanel (soft spot)
• Vomiting
• Difficulty feeding
• Extreme sleepiness or irritability
• Downward gaze (sunsetting eyes)
• Seizures

Symptoms in Older Children and Adults

• Headaches
• Visual disturbances
• Nausea and vomiting
• Coordination and balance issues
• Cognitive or memory problems
• Urinary incontinence

Treatment usually involves surgical placement of a shunt, a device that diverts excess fluid to another part of the body where it can be absorbed.

2. Nerve Damage

When tumors occur in the spinal cord, surgery may pose a risk to nearby nerves, which can result in:

• Paralysis or muscle weakness
• Chronic pain
• Loss of sensation
• Bowel or bladder dysfunction

Physical therapy and assistive devices can help patients manage these side effects, although some may be permanent.

3. Cognitive and Developmental Effects

Radiation and chemotherapy can affect brain function, especially in young children whose brains are still developing. Potential effects include:

• Learning disabilities
• Slowed growth
• Hormonal imbalances
• Emotional or behavioral changes

Educational support, counseling, and hormone therapy may be recommended in such cases.

---

Living with Anaplastic Ependymoma: Coping and Support

Receiving a diagnosis of anaplastic ependymoma can be overwhelming. While the road ahead may include surgery, radiation, and rehabilitation, there are practical steps and emotional resources that can help patients and families cope.

1. Assemble a Multidisciplinary Care Team

Choose a cancer center with experience in treating brain and spinal tumors. A team approach may include:

• Neurosurgeons
• Oncologists
• Radiation specialists
• Neurologists
• Physical and occupational therapists
• Social workers and mental health professionals

2. Seek Emotional Support

Dealing with cancer affects more than just the body—it takes an emotional toll. Support groups, individual therapy, or family counseling can help everyone involved manage stress, grief, and uncertainty.

3. Plan for Long-Term Monitoring

Even after successful treatment, regular follow-up appointments are essential. These typically include:

• MRI scans to check for recurrence
• Neurological exams
• Bloodwork to monitor overall health and side effects

4. Consider Clinical Trials

Given the rarity of anaplastic ependymomas, patients may benefit from participating in clinical trials exploring new treatment methods, including immunotherapy or targeted drug therapies.

Clinical trials offer access to cutting-edge treatments that might not be available elsewhere and also contribute to advancing medical knowledge about this rare condition.

---

Summary

Anaplastic ependymoma is a rare but serious type of brain or spinal cord tumor that tends to grow quickly and behave aggressively. Classified as a grade 3 tumor, this cancer often requires a multi-pronged approach involving surgery, radiation therapy, and sometimes chemotherapy.

Symptoms depend heavily on the tumor’s location and can include headaches, back pain, vision problems, muscle weakness, and coordination issues. The outlook varies based on factors such as age, tumor location, extent of surgical removal, and response to treatment.

While treatment can be complex and emotionally taxing, early diagnosis, expert care, and a supportive network can improve both prognosis and quality of life. With continued research and evolving therapies, there is hope for better outcomes for those facing this rare disease.