Anaplastic Ependymoma

Fact: Ependymomas can be found in children and adults

What is Anaplastic Ependymoma?
"Anaplastic ependymoma is a type of malignant ependymoma tumor commonly found in young patients. These tumors grow rapidly and many people who develop them will experience complications or even die. This is especially true if the tumor is not caught and treated early. The majority of these growths, although rare, appear in children and adults that are younger than 25 years old. Tumors like this can appear in older adults, but are much more rare.

Ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymoma is the fourth ventricle. Rarely, ependymoma can occur in the pelvic cavity.

Syringomyelia can be caused by an ependymoma. Ependymomas are also seen with neurofibromatosis."
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For those with ependymoma, the overall 5-year relative survival rate is 81.86%. 5-year relative survival rates are highest for those aged 20-44 years (90.8%), and decrease with increasing age at diagnosis with a 5-year relative survival rate of 61.9% for those aged 75+ years.

Although ependymomas are the third most common type of brain tumor in children (following astrocytoma and medulloblastoma), they are relatively rare, with approximately 200 cases diagnosed in the US each year in children and adults less than 25 years of age. Ependymomas account for 6-12% of brain tumors in children less than 18 years of age, but 30% of brain tumors in children less than 3 years of age 1. The average age at diagnosis is 4-6 years old. The underlying cause and basic biology of these tumors is not fully understood, and the optimal staging and treatment of ependymomas in children requires more study.
What are the types of Anaplastic Ependymoma?
There are numerous types of Ependymoma tumors that may develop on various parts of the cranium or the spinal cord. These include:
  • Myxopapillary ependymoma (MPE) – These develop mostly in the conus medullaris and the filum terminale of the spinal cord.
  • Sub-ependymoma – These generally occur in fourth ventricle but may also develop in the cervical spinal cord and the septum pellucidum
  • Classic ependymomas
  • Sub-ependymal giant cell astrocytoma
  • Anaplastic ependymoma – These usually occur in the posterior fossa.
  • Ependymoblastoma – These generally develop within the supratentorial compartment.
  • Extraspinal ependymoma or extradural ependymoma
  • Tanycytic ependymoma – These affect the spinal cord.
  • Intramedullary ependymoma – These occur in the spinal cord.

Grades and Diagnosis

DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.
  • Low Grade: Grade I or II means that the tumor cells are the closest to normal.
  • High Grade: Grade III or IV means that these are the most aggressive tumors.
The main issue with DIPG is that most of these tumors are not classified by grade because surgery to obtain tissue by biopsy or to remove the tumor is not safe because of the location of the tumor. When these tumors are biopsied, they are usually grade III or grade IV tumors, which tend to behave very aggressively. Most of the tumors are diagnosed by their appearance on MRI.
Symptoms of Analplastic Ependymoma
​Symptoms for Anaplastic Ependymoma vary widely and many are also common in other conditions or disorders. Please be sure to check with your doctor to ensure an accurate diagnosis and treatment.
  • Sciatica
  • Nausea
  • Seizures
  • Scoliosis
  • Paralysis
  • Vomiting
  • Dysphagia
  • Drowsiness
  • Hemiparesis
  • Loss of appetite
  • Lower back pain
  • Severe headache
  • Speech problems
  • Memory problems
  • Difficulty in sleeping
  • Bilateral Babinski sign
  • Temporary visual loss
  • Weakness of the limbs
  • Uncontrollable twitching
  • Temporary loss of memory
  • Stiffness or pain in the neck
  • Inadequate bladder control
  • Raised intracranial pressure
  • Problems with coordination
  • Development of hydrocephalus
  • Nystagmus or jerky eye movements
  • Obstruction in the flow of the cerebrospinal fluid
  • Temporary inability to identify or distinguish various colors
  • Seeing horizontal or vertical lines under the presence of bright light

CT scan or MRI is used in the initial evaluation of children suspected of having a tumor in the brain. MRI is usually preferred because of the added detail to the images and the absence of radiation exposure. Although ependymomas have a suggestive appearance on these scans, biopsy or surgical removal of tumor tissue for microscopic evaluation is necessary to make a definitive diagnosis. Evaluation of cerebrospinal fluid may be performed after surgery to detect tumor spread through the cerebrospinal fluid. Spine MRI is also usually performed.
Want to learn more?

The following organizations and/or websites offer additional information and education about Anaplastic Ependymoma. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.

Cancer Center Treatments of America
Cern Foundation
Dana-Farber Cancer Institute
Ependy Kids
Metro Health
Wise Geek Health
Weill Cornell Brain & Spine Center