Treatment for Childhood Ependymoma

Executive Summary

Tumor in children is a rare event. Hence, doctors and specialists may find it challenging to devise a treatment plan for ependymoma unless they know what has worked in other children’s cases of tumor conditions. Most (about 60%) childhood tumors are treated as part of clinical trials. A clinical trial refers to a detailed research study that focuses on testing new approaches to treat better and cure disease. Sometimes the treatment strategies approved in a clinical trial must be much better than the already existing treatment to treat a particular disease condition. The standard treatment approach includes surgery, radiation therapy, and chemotherapy. Palliative care is provided to the patients to cope with mental, physical or financial issues while involving medication, nutritional changes, emotional and spiritual support and other relaxation therapies. Parents or guardians of the affected child are urged to seek the help and guidance of their doctors to arrive at the apt clinical trial or consider all possible treatment options to cure the condition. Also, note that the safety and health of all children participating in clinical trials are closely monitored and evaluated.

Treatment for Childhood Ependymoma

Tumors in children are a rare event. Hence doctors and specialists may find it challenging to devise a treatment plan of ependymoma unless they know what has worked in the case of tumor condition in other children. This is why most (about 60%) childhood tumors are treated as part of clinical trials. A clinical trial refers to a detailed research study that focuses on testing new approaches to treat better and cure disease. Sometimes the treatment strategies approved in a clinical trial must be much better than the already existing treatment to treat a particular disease condition. Parents or guardians of the affected child are urged to seek the help and guidance of their doctors to arrive at the apt clinical trial or in considering all possible treatment options to cure the condition. Also, note that the safety and health of all children participating in clinical trials are closely monitored and evaluated.

It is always ideal for treating children with brain tumors at a specialized cancer center so that the new treatment methods of ependymoma can be made to use effectively. Also, the healthcare professionals in these specialized centers will have extensive experience handling childhood tumors and be aware of the latest studies and research. A pediatric oncologist is a doctor who specializes in treating children with cancer. In case specialized pediatric cancer centers are not available, general cancer centers with experienced pediatric specialists can be an option.

Doctors use different procedures and techniques to treat children with ependymoma tumors. Doctors use the standard care (best possible treatment option) available to treat such tumors. A multidisciplinary team will consist of doctors and specialists to carry out the treatment. In addition to treatment of ependymoma, the healthcare team will also focus on providing care and support to the children and their family members. Besides oncologists and other disease specialists, the healthcare team will include a child life specialist, nurses, physical and occupational therapists, dieticians, counsellors and social workers. Hospitals may also provide other special activities and programmes to help patients and their families cope with reality.

Described below is a list of available treatment options to treat childhood ependymoma tumors. Treating and curing the symptoms and side effects is an important part of tumor treatment and care.

The healthcare team will consider the following aspects before deciding the apt treatment strategy for a patient’s ependymoma condition.

• The tumour size.
• The tumour location
• Its stage and grade
• Whether the tumour growth is causing any signs or symptoms
• Probable side effects of treatment
• Child’s overall health condition, age and guardians’ preferences.

Parents or guardians of the affected child should take time and learn about all the available treatment options for childhood ependymoma tumors and be sure to ask questions and clear doubts about unclear and appears complex. Ask your child’s doctor about the aim of each treatment and what outcome you can expect while your child receives the treatment. This is what is called “shared decision making.” Shared decision making helps the patients to have informed choices regarding treatment. This will help the healthcare team choose and plan a treatment strategy that best fits the patient’s aims and expectations.

Surgery as Treatment of Ependymoma

Surgery is the primary treatment procedure for treating children with ependymoma brain tumors. Surgery refers to removing the tumor growth and some surrounding healthy tissues during an operation. In the case of tumors in the brain or spine, an experienced neurosurgeon performs the surgery.

Brain surgeries require the removal of a part of the skull, and the procedure is called a craniotomy. Once the surgeon removes the tumor, the patient’s bone is used to cover the skull opening made for surgical purposes. 

Today, rapid advancements have happened in brain surgeries for removing tumors. For instance, there are better imaging tests that provide surgeons more clarity regarding the tumor, thereby equipping them to better plan and perform the surgery. Also, the use of cortical mapping enables doctors to identify specific areas of the brain that control the senses, motor skills, and language better.

The primary goal of surgery is to obtain a tissue biopsy to diagnose Ependymoma and remove as much tumor growth as possible ^​1​. The disease prognosis of children with Ependymoma depends on to what extent the tumor can be resected- fully or partially. At times, more than one surgery is required for the complete resection of the tumor growth.

Post-surgery, the doctor will decide whether additional treatment for ependymoma is required based on some criteria and factors. The following are the criteria:

• The amount of tumor growth removed.
• The child’s age
• Whether the disease has spread or metastasized to another spot.

In some cases, the surgical removal of the tumor appears impossible as sometimes the tumor cannot be reached or would be near a vital structure. Such tumors which cannot be removed via surgery is termed inoperable. In such cases, the doctor will recommend other treatment options like chemotherapy or radiation therapy for eliminating the tumor mass.

Surgical procedures for treating ependymoma tumors can cause a range of side effects. Parents and caretakers of the child should talk with their doctor and understand the pros and cons of each surgical procedure. They should be well aware of the possible side effects of the ependymoma treatment and how the healthcare team will help your child manage and relieve them.

Radiation Therapy

Radiation therapy involves using high-energy x-rays or other particles to destroy the tumor cells. A radiation oncologist is a doctor who specializes in providing radiation therapy to cancer patients.

In treating ependymoma brain tumors, radiation therapy is usually used after surgery ^​2​. If the tumor growth has not metastasized, radiation therapy is directly aimed at the tumor. If the tumor has metastasized, radiation therapy is directed at the whole brain and spine. Radiation procedures can also be used in cases where a surgical resection of the tumor is impossible. Earlier, there were concerns about using radiation therapy to treat children younger than three years of age due to the range of side effects it can cause. But many recent studies and clinical trials have shown that focal radiation therapy is effective and safe to treat Ependymoma in children as young as one-year-old.

External-beam radiation therapy is the most common form of radiation therapy. It is a type of radiation given from a machine outside the body. Radiation therapy consists of x-ray particles (also called photons) or protons. A radiation therapy schedule or regimen for Ependymoma usually consists of around 30 to 33 treatments or therapies given over 6 to 7 weeks.

Radiation therapy can cause a few to several side effects. Some of them are mild skin reactions, fatigue, loose bowel movements, upset stomach etc. Most of these side effects go away once the active treatment finishes.

Chemotherapy

Chemotherapy or chemo procedure refers to drugs or medications to eliminate cancer cells. These drugs operate by preventing the cancer cells from growing, dividing and generating more cells. But normal cells also grow and divide, and chemo procedure can affect these cells, causing a range of side effects.

Chemotherapy is mainly used to slow down or stop the growth of tumors. Chemo medications can also be given before a surgical procedure to shrink or eliminate any remaining part of the tumor after surgery. In most ependymoma cases, a chemo procedure alone is not effective in eliminating the tumor. Hence chemotherapy is often combined with radiation therapy or surgery to get better results. Many clinical studies evaluate the efficiency of a chemo regimen after surgery and radiation therapy in treating ependymoma brain tumors ^​3​.

For children, chemotherapy would be given either by a pediatric oncologist or a medical oncologist. A systemic chemo regimen is directly given into the bloodstream to reach tumor cells throughout the body. Chemo drug can be given intravenously, that is, by placing an IV (intravenous) tube in the patient’s vein and using a needle; or in the form of a capsule or pill to be swallowed orally.

A chemo regimen generally consists of several cycles given over some time. A patient may receive a single type of drug or a combination of different medications, all given simultaneously.

Chemotherapy can cause many side effects. These side effects depend on the type of drug and the dose used. Some side effects include nausea and vomiting, fatigue, hair loss, risk of infection, loss of appetite, and diarrhoea. These side effects usually go away once the ependymoma treatment finishes. In some rare cases, chemotherapy can cause long-term problems that can affect the kidneys, heart and can even cause cancer.

Chemotherapy medications are continually evaluated. Many clinical trials are testing different chemo medications to treat different brain tumors. Parents should talk with their child’s doctor to learn more about the chemo drugs prescribed for their child, what outcome they should expect, and the possible side effects of treatment. Also, talk about how these chemo drugs may interact with other medications.

Physical, emotional, and social effects of a brain tumour like Ependymoma

Brain tumors like Ependymoma can cause many physical, emotional and social effects. Cancer care doesn’t end with active treatment of ependymoma. Post-treatment care is as critical as active treatment. Palliative or supportive care focus on managing and relieving symptoms and side effects associated with cancer treatment. Palliative care is carried out along with the active treatment strategy, which helps in improving the treatment efficiency by slowing the cancer growth or destroying it.

Palliative care focuses on improving a patient’s condition by helping them to manage and relieve symptoms. The care extends support not just to the patients but also to their loved ones. It also focuses on the non-medical needs of cancer patients, like managing finances. Palliative care is independent of the age, cancer stage or type of patient. Palliative care works best when it is begun right after a cancer diagnosis. This helps prevent the cancer condition and its symptoms and side effects from aggravating. Palliative focuses on improving the comfort and quality of a patient’s life. Palliative care enhances the effectiveness of active treatment. Sometimes a patient may receive chemotherapy, surgery or radiation therapy as palliative care.  

Parents or guardians of children with ependymoma tumors should talk with their child’s health care team about the aim of each treatment option, about the probable side effects and the available palliative care options before the active treatment begins.

The health care team will enquire about your child’s medical history, symptoms and side effects during treatment of ependymoma. Be sure to communicate whatever your child is feeling to the doctor. This helps them treat any symptoms or side effects that the child is experiencing with ease. This will eventually help prevent your condition from becoming worse. 

Remission and the chance of cancer recurrence

Remission refers to a stage where there are no signs or symptoms of cancer in the body. The condition can be called NED or “no evidence of disease” in the body. Remission can be permanent or temporary. Cancer patients in remission are constantly worried about a probable cancer recurrence. Patients must continue tests, scans, physical examinations even after successful treatment of ependymoma to look for recurrence. Parents or guardians of children with ependymoma brain tumors should talk with your child’s doctor about the chance of the tumor condition recurring back in your child’s body. If cancer recurs in the same place as before, it is called local recurrence, and if it happens nearby the origin point, it is called a regional recurrence. If it recurs in some distant place in the body, it is called distant recurrence.

The doctor will check for any signs of tumor recurrence. And if the tumor growth is back in the body, the doctor will list out the possible treatment options of ependymoma. In most cases, the treatment procedures for recurrent tumors include surgery, chemotherapy etc., but they may be given in different combinations.

Many clinical trials are testing the viability of various treatment options for dealing with recurrent brain tumors. Parents are encouraged to talk with their child’s doctor about any such possibilities. Whatever the treatment plan be, palliative care will always be an essential part that will primarily focus on managing and relieving symptoms and side effects of treatment.

When a tumor recurs back in the body or the disease worsens, it is quite natural and entirely ok for patients and their loved ones to feel upset and be in extreme fear. In such cases, both the patients and their caretakers are encouraged to talk to the healthcare team or counsellors or seek support services or groups to cope with the circumstances.   

What if the treatment of ependymoma does not work?

Cancer recovery may not be successful at times. The disease condition is advanced or terminal if it cannot be controlled or cured. Most ependymoma childhood tumors are treated successfully, but there may be some that cannot be fixed or cured entirely, and this condition is called advanced tumor condition.

Any cancer stage can be stressful to both patients and their loved ones. A diagnosis that states an advanced, aggressive tumor can cause even more stress and make people hopeless and uncertain about their lives. Parents or guardians of children suffering from ependymoma brain tumors should have open, honest communications with their doctor or health care team about their concerns, feelings, anxieties and preferences. Health care team members are skilled and will support the patients and their caregivers. They will focus on improving the comfort and quality of the patient’s life by providing them physical, mental and financial support.

Children diagnosed with advanced-stage tumors whose expected survival chance is less than six months can consider hospice care. Hospice care focuses on providing the best possible quality care for patients whose survival chance is low. Patients and their families can talk to the health care team about the available hospice options, including a unique hospice center, hospice care at home or any other health care location. People can also avail nursing care options.

With proper nursing care and appropriate equipment, we can even make a hospital environment at home. This helps the patient to stay at one’s home comfortably. Children would like to be engaged in activities. They would be happy to attend school on days they feel well or keep up with other social connections and activities. The parents and the healthcare can design an appropriate range of activities for the child. Parents or guardians and the healthcare team should also focus on the patient being extremely comfortable physically and free from any pain as part of end-of-life care.

The death of a person or child is an immense tragedy. Losing a person to cancer can shatter family members and loved ones. At this point, family members may require support to cope with the situation. Family members of children who died due to grave diseases like Ependymoma can seek help at the pediatric treatment centers. Most of these centers have professional support groups and staff to help people cope and face reality with courage.

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