Anaplastic ependymoma is a rare, fast-growing tumor in the brain or spinal cord. It’s most common in young children and tends to have a poor outlook. Prompt diagnosis and treatment is essential.

An ependymoma is a type of central nervous system (CNS) tumor that grows from ependymal cells in the brain or spinal cord. The term “anaplastic” describes fast-growing cells that have an unusual shape. Ependymal cells are the cells that line the fluid-filled spaces of the CNS.

Although anaplastic ependymoma can affect anyone of any age, it’s most common in babies and toddlers under the age of 4. Among this age group, tumors are typically located in the brain. Among adults with anaplastic ependymoma, tumors are more commonly found in the spinal cord.

This article reviews symptoms, diagnosis, and treatment for this type of cancer.

Ependymoma grades

Doctors classify ependymomas as grades 1, 2, or 3.

Grades 1 and 2 ependymomas are low-grade tumors, meaning they grow slowly.

Anaplastic ependymomas are grade 3, meaning they grow rapidly and often have a poorer outlook.

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Symptoms of anaplastic ependymoma vary according to a person’s age and the size and location of the tumor.

Among infants, some signs can include:

Among children and adults, some signs can include:

  • difficulty speaking
  • feeling tired
  • feeling unwell
  • headaches
  • incontinence
  • irritability
  • pain in the neck, shoulders, or upper back
  • poor balance
  • poor coordination
  • seizures
  • vision problems
  • weakness in the arms or legs

When to contact a doctor

The symptoms of anaplastic ependymoma aren’t specific to this type of tumor. A range of other illnesses can cause them. Many of those conditions aren’t serious.

Still, it’s a good idea to contact a healthcare professional if you have health concerns, particularly if you have an infant or young child with unexplained symptoms.

A family doctor or pediatrician can help determine whether additional testing is required.

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Doctors typically use brain scans, including magnetic resonance imaging (MRI) and computed tomography (CT), to diagnose anaplastic ependymoma tumors. People with anaplastic ependymoma usually need a whole spinal cord MRI as the tumor can involve areas within the brain and spinal cord.

Following a brain scan, your doctor might suggest a lumbar puncture, also known as a spinal tap, to test you or your child’s cerebrospinal fluid for cancer cells.

Finally, doctors often use surgery to confirm an anaplastic ependymoma diagnosis. A brain surgeon will extract some or all of the tumor, and a brain cancer specialist will examine the tissue to see if it’s anaplastic ependymoma.

The most common treatment for anaplastic ependymoma is a craniotomy, a type of brain surgery.

Before or after surgery, a doctor may recommend radiation therapy, chemotherapy, or both. The treatments may help shrink the tumor before surgery or destroy tumor cells not removed during surgery.

There is a risk of long-term side effects for children due to radiation, so a doctor may recommend limiting the radiation dose.

Some people may require additional treatments to help with symptoms such as fluid buildup, pain, and seizures.

Anaplastic ependymoma is an aggressive cancer that requires swift treatment to minimize complications. It tends to have a lower survival rate than other types of ependymal tumors.

According to a 2020 study that analyzed survival data from 2000 to 2016 in the United States, the outlook for people with anaplastic ependymoma depends on:

  • Age: Children under age 14 may have a more favorable outlook than adults for this type of tumor, but other research suggests the opposite is true.
  • Extent of surgery: The outlook is more favorable when a surgeon can completely remove the tumor.
  • Tumor location: Spinal cord tumors are linked to higher chances of survival than brain tumors, particularly those in the upper (supratentorial) region.
  • Race: African Americans had a lower survival rate than white people, likely due to racial disparities in healthcare.
  • Tumor recurrence: When ependymoma comes back following treatment, the long-term outlook is generally poor.

What is the life expectancy for someone with anaplastic ependymoma?

According to a 2017 study, about one-third of children with anaplastic ependymoma live for 10 years or more after diagnosis.

The 5-year relative survival rate for adults with any kind of ependymoma ranges from 87–92%, according to the American Cancer Society, although anaplastic ependymomas tend to have a poorer outlook.

Prompt diagnosis and treatment and regular follow-ups may help improve your outlook with anaplastic ependymoma.

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It’s not clear what causes anaplastic ependymoma.

The authors of a 2023 review cite studies linking ependymoma tumors to specific genetic mutations. These mutations cause cells to multiply and spread quickly.

In general, though, more research is needed to understand what causes this type of CNS tumor.

Although anaplastic ependymoma can affect anyone of any age, it’s most common in children under age 4.

It’s still rare, though. In the United States, there are only 0.43 cases for every 100,000 children under age 4.

Other factors associated with a slightly increased risk for anaplastic ependymoma tumors include:

Keep reading for answers to common questions about anaplastic ependymoma.

What is the survival rate for pediatric anaplastic ependymoma?

In a 2017 study, 57% of children with anaplastic ependymoma were still alive 5 years after diagnosis, and 35% were alive after 10 years.

How common is anaplastic ependymoma in adults?

Anaplastic ependymoma is very rare among adults. According to a 2020 study, only 948 people over the age of 15 received an anaplastic ependymoma diagnosis between 2000 and 2016 in the United States.

The authors of a 2018 study report that ependymal tumors account for 1.7% of all CNS tumors in people of all ages.

What does anaplastic ependymoma with ZFTA mean?

Ependymal tumors are categorized according to their location and molecular characteristics. Anaplastic ependymoma with ZFTA refers to tumors in the supratentorial region of the brain that also exhibit ZFTA gene irregularities.

Is anaplastic ependymoma curable?

Anaplastic ependymoma may be curable, but usually only when surgery can completely remove the tumor. But even with complete removal, it can reoccur.

Anaplastic ependymoma is a type of CNS tumor that most commonly affects young children. It’s rare, with only a few hundred people in the United States receiving a diagnosis each year.

The symptoms of anaplastic ependymoma can resemble several other conditions, so it’s important to see a doctor to learn more.