7 Key Signs and Symptoms of Ependymoma You Should Know

Ependymoma is a rare type of tumor that develops in the brain or spinal cord, originating from ependymal cells that line the ventricles of the brain and the center of the spinal cord. These tumors can occur at any age but are most commonly diagnosed in young children and adults. Understanding the symptoms of ependymoma is crucial for early detection and timely medical intervention.

The symptoms of ependymoma vary significantly depending on the tumor’s location, size, and rate of growth. When ependymomas develop in the brain, they often block the flow of cerebrospinal fluid (CSF), leading to increased intracranial pressure. Spinal ependymomas, on the other hand, may compress the spinal cord and nerves, causing different manifestations. Recognizing these warning signs can make a significant difference in outcomes.

Below are the most common symptoms associated with ependymoma that warrant immediate medical attention:

1. Persistent Headaches

Headaches are one of the most frequently reported symptoms of ependymoma, particularly when the tumor is located in the brain. These headaches occur due to increased intracranial pressure caused by the buildup of cerebrospinal fluid when the tumor blocks its normal flow through the ventricles.

The headaches associated with ependymoma typically have distinct characteristics:

• Often worse in the morning upon waking
• May be accompanied by vomiting or nausea
• Progressive in intensity and frequency over time
• May not respond well to over-the-counter pain medications
• Can be exacerbated by activities that increase intracranial pressure, such as coughing or bending over

Unlike tension headaches or migraines, these headaches tend to worsen progressively and become more persistent as the tumor grows. Parents should be particularly vigilant if their child complains of frequent morning headaches combined with other neurological symptoms.

2. Nausea and Vomiting

Unexplained nausea and vomiting are common symptoms of brain ependymomas, often occurring alongside headaches. This symptom is directly related to increased pressure within the skull and irritation of the brain’s vomiting center.

Key characteristics of this symptom include:

• Vomiting that occurs without nausea, particularly in the morning
• Projectile vomiting in some cases
• Not related to food intake or gastrointestinal issues
• May provide temporary relief from headache pressure
• Persistent despite antiemetic medications or dietary changes

This type of vomiting is neurological in origin rather than gastrointestinal, which is why it often doesn’t respond to typical remedies for upset stomach. When vomiting occurs repeatedly without an obvious cause, especially in conjunction with headaches, it should be evaluated by a healthcare professional.

3. Balance and Coordination Problems

Difficulty with balance, coordination, and walking is particularly common when ependymomas develop in or near the cerebellum, the part of the brain responsible for coordinating movement and maintaining balance. This symptom can significantly impact daily activities and quality of life.

Manifestations of balance and coordination issues include:

• Unsteady gait or difficulty walking in a straight line
• Frequent falls or stumbling without apparent cause
• Difficulty with fine motor skills such as writing or buttoning clothes
• Clumsiness when reaching for objects
• Inability to perform tasks requiring hand-eye coordination
• Tremors or shaking of the hands

In children, these symptoms might be mistaken for normal developmental clumsiness, but progressive worsening of coordination should raise concern. Adults may notice they’re dropping objects more frequently or having trouble with activities that previously came easily to them.

4. Vision Changes and Problems

Visual disturbances are another significant symptom of ependymoma, resulting from increased intracranial pressure affecting the optic nerves or direct tumor pressure on visual pathways. These changes can range from mild to severe and may affect one or both eyes.

Common vision-related symptoms include:

• Blurred or double vision (diplopia)
• Difficulty focusing on objects
• Loss of peripheral vision
• Involuntary eye movements (nystagmus)
• Seeing halos around lights
• Temporary vision loss or “graying out” when changing positions
• Papilledema (swelling of the optic disc visible during eye examination)

Parents should pay attention if children complain about seeing double, hold books very close to their face, or frequently squint. Adults may notice difficulty reading, driving, or performing tasks requiring clear vision. An ophthalmologist may detect optic nerve swelling during a routine eye exam, which can be an early indicator of increased intracranial pressure.

5. Seizures

Seizures can occur when an ependymoma irritates or disrupts normal electrical activity in the brain. While not present in all cases, seizures are a serious symptom that requires immediate medical evaluation. The type and severity of seizures can vary depending on the tumor’s location.

Seizures associated with ependymoma may manifest as:

• Focal seizures affecting one part of the body
• Generalized tonic-clonic seizures (grand mal) with loss of consciousness
• Absence seizures with brief lapses in awareness
• Unusual sensations, smells, or tastes (auras) preceding a seizure
• Temporary confusion or disorientation after the episode
• Muscle jerking or twitching in specific body parts

First-time seizures in individuals with no previous seizure history should always be investigated thoroughly, especially when accompanied by other neurological symptoms. Even brief seizure-like episodes or “staring spells” warrant medical attention.

6. Back Pain and Weakness

When ependymomas develop in the spinal cord, back pain and progressive weakness are hallmark symptoms. These tumors can compress the spinal cord and nerve roots, leading to pain and neurological deficits that affect the areas of the body served by those nerves.

Characteristics of spinal ependymoma symptoms include:

• Persistent back or neck pain that worsens over time
• Pain that radiates down the arms or legs
• Progressive muscle weakness in the extremities
• Difficulty walking or changes in gait
• Numbness or tingling in the arms, legs, or torso
• Loss of bowel or bladder control in advanced cases
• Muscle stiffness or spasticity

Unlike mechanical back pain from muscle strain or poor posture, pain from spinal ependymomas tends to be constant, progressive, and may worsen at night or when lying down. Weakness typically develops gradually and may affect both sides of the body symmetrically or asymmetrically depending on tumor location.

7. Changes in Mental Status and Behavior

Ependymomas in the brain can affect cognitive function, personality, and behavior, particularly when they involve the frontal lobes or cause significant increases in intracranial pressure. These changes may be subtle initially but typically become more pronounced as the tumor grows.

Mental and behavioral changes may include:

• Difficulty concentrating or paying attention
• Memory problems or forgetfulness
• Personality changes or mood swings
• Irritability or aggression uncharacteristic of the person
• Decreased academic or work performance
• Confusion or disorientation
• Excessive sleepiness or fatigue
• Loss of interest in previously enjoyed activities
• Developmental delays or regression in children

In children, teachers or caregivers might notice declining school performance, behavioral problems, or social withdrawal. Adults may experience difficulty with complex tasks at work or changes in social interactions. Family members are often the first to notice these subtle personality or cognitive changes.

Main Causes of Ependymoma

The exact cause of ependymoma remains largely unknown, and in most cases, no specific risk factor can be identified. However, researchers have identified several factors that may be associated with the development of these tumors:

Genetic Factors

While most ependymomas occur sporadically without any family history, certain genetic conditions increase the risk of developing these tumors. Neurofibromatosis type 2 (NF2), a hereditary disorder, is associated with an increased incidence of ependymomas, particularly spinal cord tumors. Some studies have also identified specific genetic mutations and chromosomal abnormalities in ependymoma cells, though these are typically acquired rather than inherited.

Age and Demographics

Ependymomas can develop at any age, but they show two peak incidence periods: in young children (particularly those under 5 years old) and in adults in their 30s and 40s. The location of tumors also varies by age, with intracranial ependymomas being more common in children and spinal ependymomas more frequent in adults.

Previous Radiation Exposure

There is some evidence suggesting that previous radiation therapy to the head or spine, particularly during childhood, may slightly increase the risk of developing ependymomas years later. However, this accounts for only a small percentage of cases.

Unknown Environmental Factors

While various environmental factors have been studied, no definitive environmental causes have been established for ependymoma. Unlike some other cancers, lifestyle factors such as diet, smoking, or occupational exposures have not been definitively linked to ependymoma development.

It’s important to note that in the vast majority of cases, ependymomas develop without any identifiable cause or risk factor, and there is typically nothing that could have been done to prevent them.

Frequently Asked Questions

What is the difference between ependymoma and other brain tumors?

Ependymomas specifically arise from ependymal cells lining the ventricles of the brain and central canal of the spinal cord, whereas other brain tumors originate from different cell types. Ependymomas have distinct microscopic features, genetic profiles, and tend to occur in characteristic locations within the central nervous system.

Can ependymoma symptoms come and go?

While some symptoms may fluctuate in intensity, ependymoma symptoms typically follow a progressive pattern, gradually worsening over time as the tumor grows. Temporary relief might occur after vomiting (which temporarily reduces intracranial pressure) or with certain position changes, but the underlying symptoms generally persist and worsen without treatment.

How quickly do ependymoma symptoms develop?

The timeline for symptom development varies considerably depending on the tumor’s growth rate and location. Some ependymomas are slow-growing and may produce subtle symptoms over months or even years, while others grow more rapidly, causing noticeable symptoms within weeks to months. Generally, symptoms become more pronounced and frequent as the tumor enlarges.

Are ependymoma symptoms different in children versus adults?

The fundamental symptoms are similar across age groups, but presentation may differ. Children more commonly have infratentorial (posterior fossa) brain tumors causing balance problems, vomiting, and headaches, while adults more frequently develop spinal ependymomas causing back pain and weakness. Children may also have difficulty articulating their symptoms, making observation of behavioral changes crucial.

Can ependymomas be detected before symptoms appear?

Ependymomas are rarely detected before symptoms develop because there are no routine screening tests for these tumors in the general population. Occasionally, they may be found incidentally on brain or spine imaging performed for unrelated reasons. People with neurofibromatosis type 2 may undergo more frequent monitoring that could detect tumors earlier.

When should I see a doctor about potential ependymoma symptoms?

You should seek medical evaluation promptly if you or your child experiences persistent headaches that worsen over time, especially when combined with vomiting, vision changes, balance problems, or seizures. New neurological symptoms such as weakness, numbness, or changes in coordination should always be evaluated. While these symptoms can have many causes less serious than ependymoma, early medical assessment is important for proper diagnosis.

Do all ependymomas cause symptoms?

Most ependymomas eventually cause symptoms due to their location in confined spaces (brain ventricles or spinal canal) where even small tumors can block fluid flow or compress neural tissue. However, very small or slow-growing tumors might not produce noticeable symptoms for an extended period. Once tumors reach a certain size or critical location, symptoms typically develop.

Can stress or lifestyle factors worsen ependymoma symptoms?

While stress and lifestyle factors don’t cause ependymomas, adequate rest and stress management can help with overall well-being during diagnosis and treatment. The symptoms themselves are caused by the tumor’s physical effects on the brain or spinal cord, not by external factors. However, activities that increase intracranial pressure (such as heavy lifting or straining) may temporarily worsen symptoms like headaches.

References:

• Mayo Clinic – Ependymoma
• National Cancer Institute – Childhood Ependymoma Treatment
• Johns Hopkins Medicine – Ependymoma
• American Association of Neurological Surgeons – Ependymoma
• American Cancer Society – Types of Brain and Spinal Cord Tumors in Adults