Ependymoma is a rare tumor that develops in the brain or spinal cord, arising from special cells that line fluid-filled spaces in the central nervous system. While it can affect people of any age, this type of tumor shows distinct patterns in children versus adults, both in where it forms and how it behaves.

Understanding Ependymoma Across Age Groups

Ependymoma represents a relatively uncommon group of tumors within the central nervous system. According to statistics from the United States, this condition affects approximately 1.7% of all brain and central nervous system tumors when considering all age groups^[6]. The median age at diagnosis sits at 44 years, though the disease appears across the entire lifespan^[6].

In children and adolescents, ependymoma takes on greater significance. It ranks as the third most common brain tumor in pediatric patients and the sixth most common brain tumor specifically in children^[2][6]. Each year in the United States, roughly 230 children and adolescents between ages 0 and 19 receive an ependymoma diagnosis^[4]. Among children, these tumors represent about 9% of all childhood brain and spinal cord tumors, translating to approximately 200 cases annually^[3][15]. The condition most frequently appears in children under age 5, though diagnosis at any childhood age remains possible^[22].

For adults, approximately 1,100 individuals receive an ependymoma diagnosis each year in the United States, making it account for less than 2% of central nervous system tumors in this age group^[4]. Adults with ependymoma tend to be diagnosed in their 30s or 40s^[5].

The location where ependymoma develops varies significantly between age groups. In children, about 65% to 75% of these tumors arise in the posterior fossa, which is the lower back region of the brain near the cerebellum and fourth ventricle^[15]. The majority of childhood cases occur in the brain rather than the spine^[4]. Conversely, adults most commonly develop ependymomas in the spinal cord rather than within the brain itself^[1][5].

What Causes Ependymoma

The underlying cause of ependymoma remains largely unknown to medical researchers. These tumors develop from ependymal cells, which are specialized cells that line the fluid-filled spaces in the brain called ventricles and the central canal of the spinal cord^[3][8]. Ependymal cells belong to a group of support cells in the nervous system known as glial cells, and they play an important role in producing cerebrospinal fluid^[4][15].

Cancer itself is considered a genetic disease, meaning it results from certain changes to genes that control how cells function. These genes may become mutated, which can increase the growth and spread of cancer cells^[3]. In the case of ependymoma, these genetic changes may occur by mistake when ependymal cells divide and replicate^[2].

Most cases of ependymoma occur sporadically, with no confirmed or identifiable cause^[5]. However, researchers have identified some connections between certain inherited genetic conditions and increased risk for developing ependymoma. People with a genetic disorder called neurofibromatosis type 2 appear to have a higher likelihood of developing these tumors^[2][3][8]. Other inherited conditions that may increase risk include Turcot syndrome and BMEN1 syndrome^[5].

Recent scientific studies have revealed that ependymomas arising from different regions of the central nervous system, while looking similar under the microscope, often follow substantially different clinical courses. This suggests that these tumors may actually represent distinct populations of cells, which would explain why tumors with the same appearance can behave so differently^[6]. Several genetic abnormalities have been discovered that correlate with ependymoma and may provide more accurate predictions about how the tumor will behave than traditional classification systems alone^[6].

Risk Factors for Developing Ependymoma

Because the exact cause of ependymoma is not well understood, doctors often cannot identify specific circumstances that increase the risk for developing this type of tumor. The disease does not follow clear patterns that would allow healthcare providers to predict who will develop it^[5].

The most clearly identified risk factors relate to certain inherited genetic conditions. Neurofibromatosis type 2 represents the most notable genetic syndrome associated with ependymoma risk^[2][3][8]. However, researchers emphasize they cannot yet say with certainty that these genetic variations definitively cause ependymomas—they are still studying this possible connection^[2].

Age itself appears to influence both the likelihood of developing ependymoma and where it will form. Young children, particularly those under age 5, represent a higher-risk group for developing brain ependymomas^[5][22]. Adults in their 30s and 40s face increased risk compared to other adult age groups, and when adults do develop ependymoma, it more commonly appears in the spinal cord^[5].

Family history of cancer or brain tumors may be relevant, as doctors will typically ask about this when evaluating a patient^[5]. However, no clear environmental exposures, lifestyle behaviors, or occupational hazards have been definitively linked to ependymoma development.

Recognizing Symptoms of Ependymoma

The symptoms a person experiences with ependymoma depend primarily on where the tumor is located, how large it has grown, and what age the patient is. Because these tumors develop in different parts of the brain or spinal cord, they create pressure on or interfere with different structures, leading to varied symptom patterns^[1][3].

Brain Ependymoma Symptoms

When ependymoma develops in the brain, it often causes symptoms by compressing surrounding structures or blocking the normal flow of cerebrospinal fluid. This blockage can lead to a condition called hydrocephalus, where cerebrospinal fluid builds up in the brain’s ventricles, creating pressure within the skull^[5][8].

People with a brain ependymoma may experience headaches, which can be persistent or severe^[1][2][3]. Nausea and vomiting frequently accompany the headaches^[1][2][3]. Vision changes, including blurry vision or other visual disturbances, may occur^[1][2]. Seizures represent another possible symptom when the tumor affects brain tissue^[1][2].

Additional symptoms can include dizziness, confusion, irritability, and problems with balance or coordination^[1][2][3]. Some people experience weakness, fatigue, loss of appetite, trouble concentrating, or changes in mood and personality^[5]. Difficulty walking, problems with coordination, and general loss of balance may develop as the tumor grows^[5].

In babies and very young children who cannot communicate their discomfort, parents might notice different signs. These can include a larger-than-usual head size, unusual sleeplessness, increased irritability or fussiness beyond what is normal, and vomiting or spitting up more frequently than usual^[2]. Young children may also fall behind on expected developmental milestones^[5].

Spinal Cord Ependymoma Symptoms

When ependymoma forms in the spinal cord, it creates a different set of symptoms related to nerve function and spinal cord compression. Back pain or neck pain often develops, which may be persistent^[2][3]. People may experience numbness and weakness in their arms, legs, or trunk^[2][3].

Muscle weakness in one or both legs can occur, sometimes affecting the ability to walk normally^[5]. Neck stiffness and neck weakness may be present^[5]. Some people develop problems controlling their bladder or bowel function, which can manifest as urinary incontinence or other bladder and bowel dysfunction^[2][3][5].

Preventing Ependymoma

Currently, there are no known methods to prevent ependymoma. Because researchers do not fully understand what causes these tumors to develop, specific prevention strategies have not been established. The condition occurs largely randomly, without clear links to modifiable lifestyle factors, environmental exposures, or behaviors that people could change to reduce their risk.

For individuals with known genetic conditions associated with increased ependymoma risk, such as neurofibromatosis type 2, genetic counseling may be valuable. These services can help affected individuals and their families understand their risks and consider appropriate monitoring strategies. However, even in these cases, no definitive prevention methods exist.

The best approach involves awareness of symptoms and seeking prompt medical evaluation when concerning signs appear. Early detection and diagnosis allow for earlier treatment, which may improve outcomes. Parents should pay attention to developmental changes in children and consult healthcare providers if worrisome symptoms develop. Adults experiencing persistent back pain, neurological changes, or other concerning symptoms should not delay seeking medical evaluation.

How Ependymoma Affects the Body

Ependymoma creates problems in the body primarily through mechanical effects—the physical presence of the tumor mass disrupting normal structures and functions. Understanding these changes helps explain why symptoms develop and why treatment is necessary.

Location and Growth Patterns

Ependymomas can form anywhere along the central nervous system, though they show preferences for certain locations. These tumors often occur near or within the ventricles in the brain and the central canal of the spinal cord—the very spaces where cerebrospinal fluid flows^[3][8]. On rare occasions, ependymomas can even form outside the central nervous system, such as in the ovaries^[3].

The tumors are classified into different types based on several factors: how the cells appear under a microscope, where the tumor is located, and what genetic changes exist in the tumor cells^[3][6]. The 2021 World Health Organization classification system identifies ten main subtypes of ependymal tumors based on anatomical site and molecular features^[15].

Healthcare providers grade ependymomas on a scale from 1 to 3 based on how quickly they grow. Grade 1 tumors grow the slowest, while grade 3 tumors grow fastest^[2]. Grade 1 and 2 ependymomas are generally noncancerous, meaning they usually grow slowly and do not spread from where they form^[2]. Grade 3 ependymomas are cancerous and grow more aggressively, spreading more quickly than lower-grade tumors^[2].

In general, ependymal tumors tend not to invade nearby tissue but instead displace it as they grow. This characteristic makes surgical removal of many ependymomas possible^[13]. However, the grade does not always perfectly predict how the tumor will behave—some ependymomas act differently than their grade would suggest^[7].

Fluid Blockage and Pressure Effects

One of the most significant ways ependymoma affects the body involves disrupting the normal flow of cerebrospinal fluid. This clear fluid surrounds and protects the brain and spinal cord, flowing through specific pathways including the ventricles. When an ependymoma grows near these passageways, it can block the fluid from flowing freely^[5].

When cerebrospinal fluid cannot drain properly, it accumulates in the ventricles, leading to hydrocephalus. This buildup creates pressure inside the skull, which can damage brain tissue and cause severe symptoms^[5][8]. The increased pressure explains many symptoms such as headaches, nausea, vomiting, and vision problems.

Spread Within the Central Nervous System

Ependymomas rarely spread outside the central nervous system to other parts of the body^[3][4][8]. However, they can spread to other areas within the central nervous system through cerebrospinal fluid^[3][4]. About 10 to 15% of ependymomas spread this way to different locations in the brain or spinal cord^[13]. The tumor cells can travel through the cerebrospinal fluid that bathes the brain and spinal cord, potentially seeding new tumor growth in distant locations^[8].

Certain types of ependymomas, particularly anaplastic ependymomas (grade 3), tend to spread more readily through cerebrospinal fluid to other parts of the brain^[2]. These higher-grade tumors are also more likely to return after treatment^[2].

Nerve and Tissue Compression

As ependymomas grow, they occupy space and push against surrounding brain or spinal cord tissue. In the brain, this compression can affect various functions depending on which areas are impacted—leading to problems with movement, sensation, cognition, or behavior. In the spinal cord, tumor growth can compress nerve roots or the spinal cord itself, causing pain, weakness, numbness, and problems with bladder or bowel control.