EPENDYMOMA

An Ependymoma is a slow-growing tumor in the brain and spinal cord. Because it originates in your brain or spinal cord as opposed to somewhere else and spreads, it is known as a primary central nervous system tumor.

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3 percent of individuals with progressive curvature may eventually experience severe problems that can include scoliosis and back pain, spinal problems, and nerve compression causing numbness, weakness, and leg pain.

Ependymoma

Ependymoma is a slow-growing tumor in the brain or spinal cord. Because it originates in your brain or spinal cord as opposed to somewhere else and spreads, it is known as a primary central nervous system tumor. Beyond your brain or spinal cord, these cancers often don’t metastasize. Children are more likely than adults to develop them as they grow. Ependymomas are intramedullary tumors, which implies that they develop within the actual spinal cord’s tissue. Including the cervical (neck), thoracic (upper back), and lumbar levels of the spinal cord, they can happen anywhere (lower back). The lower lumbar and sacral area (base of the spine, above the tailbone) is where certain ependymomas, sometimes referred to as myxopapillary filum terminals ependymomas, develop. Below the spinal cord, this structure is a thin, fibrous thread that aids in spinal cord stability. It might be risky to remove ependymomas in this region.

Types of Ependymomas

Your doctor at Southwest Scoliosis and Spine Institute will discuss the sort of ependymoma you or your child has. Based on where your tumor is located and how quickly it spreads, there are four different types.

Subependymomas (grade I)

These often develop slowly and appear close to a ventricle in the brain. Adults and elderly males experience them most frequently.

Myxopapillary ependymomas (grade II)

These most frequently affect young adults’ spinal cords. Their growth is sluggish.

Ependymomas (grade III)

The brain is where this ependymoma tumor, which is the most prevalent kind, often develops.

Anaplastic ependymomas (grade IV)

These tumors frequently have a bad prognosis and develop quickly. Additionally, they frequently grow in the region of the brain that houses the brain stem and cerebellum. The posterior fossa is the name given to this region of the brain.

Causes of Ependymoma

The average age at which a spinal ependymoma develops is 39 years old, however, roughly one in four tumors appear before the age of 18. Males are more likely than females to develop a spinal ependymoma, however, it is unclear why this is the case. Spinal ependymomas are intramedullary tumors that form inside the spinal cord, as we noted above. They can grow in a number of different locations inside the cord, though. The majority of ependymomas are present in the cervical cord, where they grow in this region in roughly 44 percent of cases. The thoracic cord alone accounts for around 26% of the development, while the cervical cord and upper thoracic chord together account for another 23%.

How Common are Ependymomas?

Nearly 22 out of every 100,000 persons, according to experts, have primary central nervous system malignancies. Ependymomas represent all main central nervous system tumor diagnoses:

  • 2% or less of adult diagnoses.
  • 4% of 15 to 19-year-old youngsters receive a diagnosis.
  • Almost 6% of pediatric diagnoses from birth to age 14

Symptoms

Ependymal cells in the spinal cord’s inner core give birth to spinal ependymomas. They progressively exert pressure on the surrounding spinal cord by slowly expanding over an extended period of time, sometimes years. This compression of the spinal cord can cause pain symptoms at the tumor site. Numbness and tingling in the arms and legs; weakness and clumsiness in the arms and legs. The size and location of the tumor affect the specific symptoms. In terms of onset and development, symptoms differ greatly.

They might be brief, sporadic, or persistently non-progressive. Since intramedullary spinal cord tumors are benign and slow-growing, they may go on for a long period without showing any symptoms. This may delay patient presentation for medical evaluation and diagnosis. Fortunately, most patients today are initially diagnosed with little or no neurological abnormalities. There are exceptions, and the onset and progression of symptoms may occur over a short period of time.

Diagnosis of Spinal Ependymomas

To determine if you have an ependymoma, the doctor could perform the following tests on you:

Physical exam

The doctor examines you and inquires about your health and past illnesses.

Neurological exam

In order to examine your spinal cord, brain, and nerves, the doctor will have you move about.

MRI

This scan takes detailed pictures of your organs. They will likely take pictures of the pigment that gathers around the cancer cells so that they are easier to see in the picture.

Spinal tap

In order to draw fluid from your spine, you will lie on your side while the doctor uses a needle. In order to look for cancers, lab technicians will examine them.

Biopsy

The only way to tell if a tumor is an ependymoma is to look for cancer cells on a small piece under a microscope. This happens during surgery. Also, the biopsy will tell your doctor about the type and grade of the tumor.

Risk Factors

Ependymoma’s origins are yet unknown. Both men and women are equally affected, and the majority of ependymomas develop between the ages of four and six. But people of all ages, races, and sexes are susceptible to developing the tumors. Ependymomas are more likely to form in people with a hereditary disorder known as neurofibromatosis type 2.

Ependymoma Treatments

Treatments for Ependymoma options include:

Ependymoma surgery to remove the tumor

To remove as much of the ependymoma as feasible, the Southwest Scoliosis and Spine Institute Orthopedic Surgeons will conduct surgery. The aim is to remove the entire tumor, but occasionally the ependymoma lies close to delicate brain or spinal tissue, making it too dangerous. Your child might not need any further therapy if the entire tumor is removed during surgery. The neurosurgeon may suggest a different procedure to attempt to remove the remaining tumor if part of it is still present. For more aggressive cancers or in cases where the tumor cannot be completely removed, other therapies, such as radiation therapy, may be advised.

Radiation therapy

High-energy radiation, such as x-rays or protons, is used in radiation treatment to destroy cancer cells. During radiation therapy, your child lies on a table and a machine moves around your child, aiming beams at precise points in the spine. Radiation therapy may be recommended after surgery to prevent more aggressive tumor recurrence or if the neurosurgeon cannot completely remove the tumor. A special technique helps the treatment irradiate the tumor cells, sparing as much of the surrounding healthy tissue as possible. Radiation treatment techniques including conformal radiation therapy, intensity-modulated radiation therapy, and proton therapy enable medical professionals to deliver radiation with care and accuracy.

Radiosurgery

Stereotactic radiosurgery, which is technically a form of radiation rather than surgery, concentrates several radiation beams on specific locations to destroy the tumor cells. When ependymoma returns after surgery, radiation, and radiosurgery may be employed.

Chemotherapy

To eliminate cancer cells, chemotherapy will also be used.  Most instances of ependymoma don’t respond well to chemotherapy. Chemotherapy is only used after other treatments have failed, such as surgery and radiation, and is still pretty much trial and error. An example of this is when the tumor returns after treatment.

Clinical trials

Clinical trials are investigations of novel therapies. You have the opportunity to try the most recent treatments thanks to this research, but it’s possible that there are unknown adverse effects. If your kid is qualified to take part in an FDA Approved research study, ask your doctor.

Targeted drug therapy

Drugs that target cancer cells are being investigated under approved FDA supervision. These could affect surrounding healthy cells less than other forms of treatment.

Recovery

Cancer treatment recovery can be a protracted and difficult process. Chemotherapy can make a person feel weak and nauseous. Sometimes after neurosurgery, patients feel worse than they did before the procedure. It’s typical to experience fatigue, confusion, weakness, and vertigo. However, if the procedure brings on the desired outcome, these other feelings will pass quickly.

Long-term effects

In rare instances, ependymoma or surgical side effects might last a long time or be late. Anywhere in the body might experience these side effects, and they may appear months or years after surgery. Heart, lung, and secondary cancer issues are a few examples of the physical long-term impacts. Anxiety, sadness, or learning challenges are possible additional long-term impacts. There may also be memory or cognitive problems. In order to check for any long-lasting effects, doctors will want to frequently follow a patient. In some cases, further examinations or tests are required to identify a problem. Different types of rehabilitation, such as physical treatment, hearing aids, or cognitive therapy, could be necessary for some persons. In addition, hormonal imbalances and developmental problems may occur in children.

There is a 65 percent cure rate if the tumor can be entirely removed. Later on, though, more ependymomas may form. It may be challenging to treat recurrent ependymomas. A myxopapillary ependymoma often has a better prognosis than a classic or anaplastic variety. The prognosis for adults is often better than that of youngsters. However, with appropriate care, roughly 82% of those with ependymomas live for at least five years. Additionally, pursuant to FDA rules and regulations there are ongoing clinical trials that evaluate novel therapies and examine ways to reduce the side effects of therapies.

 

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