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Ependymoma: A thorough review in differing treatments available through updateddiscoveries in medical imaging, radiotherapy, and proton beam therapy

  • sunshine4cancerkid
  • Aug 10
  • 24 min read

Sarah Al-Najjar, Amirah Jannati, Muhammed Imran, Maansi Sharma, Gabrielle Danso

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Contributor(s):

Amirah Jannati | Grade 11

Gabrielle Danso | Grade 12

Maansi Sharma | Grade 11

Muhammed Imran | Grade 11

Sarah Al-Najjar | Grade 11

Abstract


Ependymoma is a type of cancer that often occurs in children, and some adults. It causes

an abnormal amount of cell growth, often in the spinal cord or brain as they are Central Nervous

System (CNS) tumor Ependymomas can be cancerous as well as non cancerous. Non cancerous

ependymomas are tumors that are benign, while cancerous are malignant tumors.


Ependymoma’s are unique in the sense that while they are more commonly found in

children, adults can be diagnosed with Ependymoma as well. However, children are more likely

to have Ependymoma in the brain while adults tend to develop Ependymoma in the spinal cord,

resulting in differing treatment for both groups


This paper aims to cover the diagnosis, treatments, and medical professions relating to

Ependymoma, with an emphasis on different treatments available to patients with Ependymoma.

The selection of which treatment a patient may receive can differ based on age, diagnosis, and

preexisting health issues. Treatment for Ependymoma discussed in this paper are as follows:

Immunotherapy, radiotherapy, chemotherapy, Advanced Machinery Interventions, HER2 CAR T

cells, and other forms of treatments from clinical trials.


Additionally this paper focuses on the various different types of medical professions

involved in treating, researching, and overall dealing with Ependymoma. This includes

Neurosurgeons, Neuroradiologists, Neuropathologists, and Physical Therapists who all are a part

of the diagnostic, treatment, and recovery process for patients with Ependymoma.


Introduction

Tumors:

A tumor is an abnormal growth of cells that forms in a human body. Tumors can either be

Benign (non cancerous) or malignant (cancerous). Oftentimes, the first treatment option for

tumors is surgery.


Ependymoma:

This is the case for tumors such as Ependymoma. The prognosis for Ependymoma is

based on the type of tumor (benign/malignent), age, size, and other factors, Over all, the 5 year

survival rate of Ependymoma is close to 88%( (“Ependymoma: Diagnosis and Treatment -

Ependymoma occurs as a result of mutated genes, as well as certain genetic disorders like

Neurofibromatosis type 2. Because Ependyoma is a Central Nervous System tumor, it mostly

affects the brain and spinal cord. This keeps the Ependymoma relatively contained, however,

they may spread throughout the body through CSF (cerebrospinal fluid).

Ependymoma’s are more likely to occur in children, though anyone can get

Ependymoma. Ependymoma’s are more prevalent in white (Non Hispanic) individuals as well as

males ( (“Ependymoma: Diagnosis and Treatment - NCI”).

This paper aims to highlight different research available for patients with Ependymoma,

as well different treatments available through the advancement of medical technology and

radiotherapy.


Discussion

Why Ependymoma?

  • Ependymoma uniquely isn’t considered for chemotherapy as frequently as other cancers. While chemotherapy is minimally effective due to the sensitive nature of the locations, there are many other treatments that are undergoing research. Additionally, ependymoma has many influences such as epigenetics from DNA mutations, and each tumor is unique depending on their location, which is why it’s important to consider many therapies and treatments (Smith).

  • Ependymoma is distinctive in the sense that it is a cancer that occurs more frequently in children, but also in adults. However, this cancer tends to differ based on the age of a patient. Typically, children with Ependymoma develop tumors in the brain, and are diagnosed ages 8 and under, while adults are diagnosed typically ages 30-40 and develop tumors in the spinal cord (Yale Medicine). The difference in how Empendymoma develops and presents itself in children and adults is very important, and essential in understanding and research for pediatric cancers.

  • Ependymomas are the third most commonly found tumors in children. With its particularly difficult location being the spine and the brain stem and due to developing nervous systems in children; surgery, intervention and outcomes can be more severe. Children with ependymoma are forced to undergo aggressive treatments with uncertain long-term benefits, side effects and a high recurrence rate. Ependymomas are majorly understudied, and the goal of this research/review paper is to provide information and useful research/statistics to make a beneficial change in the diagnosis, treatment and vanquishing of ependymomas to ensure better survival and quality of life in children, along with further advances in medicine.

  • By dedicating research to ependymomas we can also gain knowledge on cutting edge technological advancements in the medical field such as proton beam therapy, introduced and widespread late into the previous century. Like the name suggests, it uses a beam of protons to destroy cancer. The advanced technology used to treat ependymomas has saved the life of countless pediatric patients and has given ependymoma’s victims hope.

  • Unlike well-known cancers, ependymomas is a rare tumor that deeply impacts the lives of children. It is such a fascinating yet upsetting concept with how the tumor is in the central nervous system and interrupts the development of both the brain and spinal cord. It is even worse for patients considering how it rejects chemotherapy leaving doctors with limited tools and solutions to find new ways to minimize the tumor, as there is no set cure. For the kids it is not about survival it is about finding joy in the little things, like laughing, walking or connectingwith friends. Getting the opportunity to explore and learn more about this tumor can enhance the common knowledge of it bringing more awareness to ependymomas.



How to Diagnose Patients with Ependymoma

Introduction to Patient Diagnosis


Overview:

When diagnosing patients with Ependymoma, there are many factors that are taken into

consideration. The signs and symptoms vary depending on the location of the Ependymoma

tumor, whether it be in the brain or spinal cord. To officially diagnose a patient, doctors must

perform: physical examinations, diagnostic testing, and performing biopsies on the tissue of the

tumor for grade determination. Grade I indicates Subependymoma, Grade II indicates

Myxopillary or Conventional Ependymoma, and Grade III indicates Anaplastic Ependymoma

(“Ependymoma: Diagnosis and Treatment - NCI”).


Challenges with Diagnosis:

Considering the rarity of Ependymoma cases amongst children, many doctors and

oncologists mistake Ependymoma with another disease. Ependymoma has many symptoms that

mock common illnesses since they are nonspecific. When performing biopsies of the tissue of

the tumor, the cells may behave differently compared to its expected cellular characteristics,

making it difficult to predict further treatment plans and the grade of the tumor

(“Ependymoma”).


Signs of Ependymoma

Signs of Ependymoma include physical indicators of the cancer that the patient presents.


Ataxia:

Ataxia, which is uncoordinated balance, gait, and clumsiness, is a common sign of

Ependymoma. This is due to the tumor exerting too much pressure onto the central nervous

system. The typical location of the tumor is near the posterior fossa, close to both the cerebellumand the brainstem. The cerebellum is responsible for movement coordination (“Childhood Ependymoma Treatment (PDQ®) - NCI”).


Loss of Bowel Movement Control:

Typically, if the tumor is on the spinal cord, it pressures the segments that directly impact

the bladder and its functions. It can affect the signals that the brain sends to the bowel to help

control its function and a reduction of bowel control. It can also lead to further problems

regarding urination and constipation (Cohen).


Speech Difficulties:

As the tumor grows in either the brain or spinal cord, it puts pressure on the parts of the

brain responsible for language processing and speech. This is through tumor interference with

the signals to send to the pterygoid and masseter muscles which assist in producing speech from

the brain. It can further lead to slurred speech and difficulties with word pronunciation (“Speech

and language difficulties”).


Cranial Nerve Palsies:

Cranial Nerve Palsies (CNP) are defined as facial muscle weaknesses. This is due to the

tumor pressurizing cranial nerves, either through physical pressure or due to hydrocephalus,

which is fluid in the brain (Varrassi et al.).


Macrocephaly:

Macrocephaly is an unusual head enlargement due to hydrocephalus. This is typically

found in younger patients of Ependymoma, and occurs because an infant's head is not fully fused

and the size of the tumor can cause the head to enlarge. There is also pressure from the tumor,

ultimately leading to a rapid increase of an infant's head (“Ependymoma”).


Seizures:

Seizures are involuntary movements, feelings, and impacted consciousness due to an

interruption in the signals in the brain. This can be related to Ependymoma as the location of the

tumor can impact the function of the brain such as blocking off signals and disrupting theelectrical activity of the brain. More specifically, Ependymomas can block cerebrospinal fluid

flow which leads to immense pressure on the nerve signals (“Ependymoma - Symptoms and

causes”).


Symptoms of Ependymoma

Symptoms of Ependymoma include feelings of the patient that the doctor doesn’t visibly see.


Headaches:

Headaches are majorly caused by increased intracranial pressure. This increased pressure

can come from the buildup of cerebrospinal fluid, leading to an accumulation in the brain,

commonly referred to as hydrocephalus (“Headaches and Hydrocephalus: Causes and Types

Explained”).


Dizziness, Nausea, and Vomiting:

With increased intracranial pressure, it triggers feelings of nausea and vomiting.

Dizziness can be caused by the impact of the tumor on the cerebellum impacting balance, which

can further trigger nausea and vomiting. Tumors can also impact hormonal levels that can cause

someone to experience increased feelings of nausea (V ogelbaum).


Vision Difficulties:

If the location of the tumor is exerting pressure onto the brainstem, it can lead to double

vision as the brainstem is responsible for eye movement and pathways. Blurred vision can be an

effect of the tumor being located close to the optic nerve, which may also lead to loss of vision

(V ogelbaum). Finally, nystagmus, or unusual rapid eye movements, can appear due to pressure

on the cerebellum affecting movement of the eyes coming from hydrocephalus (“The Types of

Vision Problems Caused by Hydrocephalus”).


Education Difficulties:

Performance at school can be a symptom of Ependymoma. This is majorly due to the

location of the tumor, as tumors in the cerebrum have the purpose of affecting problem-solving,

learning, and thinking skills (“Learning difficulties in children”).Physician and Machine


Interventions in Diagnosis

Physical and Neurological Examination:

Patients first discuss with a healthcare provider about their current signs, symptoms, and

concerns. After reviewing any histories and evaluating any predisposed risks the patient may

have, they are referred to a neurologist, where further tests are conducted that have functions

directly correlated to the brain and spinal cord (“Ependymoma”).


Magnetic Resonance Imaging (MRI):

MRIs are used to help create images to visualize the brain and spine. MRIs use radio

waves and magnets, and help to map the location and size of the tumor. From there, the

characteristics of the tumor can be determined (“Ependymoma”).


Computed Tomography (CT):

CT scans use X-rays to help find the location of the tumor and determine if the tumor has

any interference with bones in the spine (“Ependymoma”).


Biopsy:

A biopsy is a small sample of the tumor that is sent to pathology for analysis. The tissue

gets examined to help determine the important characteristics of the tumor and the types of cells

involved (“Ependymoma”).


Positron Emission Tomography (PET):

A PET scan uses metabolic activity from the brain to evaluate tumors and determine their

grade (“Ependymoma”).


Lumbar Puncture:

A lumbar puncture, also known as a spinal tap, is when a needle is inserted into the spine

to collect a sample of cerebrospinal fluid. This fluid can be used to test where in the central

nervous system the tumor has spread to (“Ependymoma”).


Medical Professions Associated with Ependymoma


Overview:

Treating Ependymoma requires a team of multiple different types of doctor in order to

properly treat the cancer. Doctors commonly associated with ependymoma treatment include

neurosurgeons, neurooncologists, neuroradiologists, neuropathologists, and physical therapists.


Neurosurgeons:

A neurosurgeon is a type of doctor that diagnoses and treats conditions that affect your

spinal cord, brain, nervous system, and nerves. Despite having a surgeon in their name,

neurosurgeons may also partake in nonsurgical roles (Cleveland Clinic). To become a

neurosurgeon requires 14-16 years of commitment to school. You must first complete your 4

years pre-med undergraduate track, then 4 years of medical school, then start about a 7 years

internship or residency. Neurosurgeons are one of the highest paid doctors in the world earning

an average salary of $721,001 in the US (Salary.com).


Neuro-oncologist and Neuroradiologists:

A neuro-oncologist and neuroradiologists are medical specialists who focus on the

diagnosis, treatment, and management of brain and spinal cord tumors. They typically use

advanced radiological techniques to carefully assess and determine the options for treatment

(George Washington Hospital). Like most other professions in the neuro field, you must have 4

years of pre-med undergrad, 4 years of medical school, and about 7 years of residency.

Neuro-oncologist typically make about $381,094 per year in the US and neuroradiologists

typically make $381,061 per year (Zip Recruiter)


Neuropathologists:

A neuropathologist is a doctor that has the role in examining tissues of the nervous

system. They examine radiologic studies along with the tissue samples to determine whether the

abnormal tissue is benign or a cancerous tumour (UPMC). Becoming a neuropathologist

typically takes about 12-14 years.You need 4 years of undergraduate pre-med study, 4 years of

medical school, 4 years of residency, and a 1-2 year fellowship in neuropathology (AmericanUniversity of Antigua). Neuropathologists typically make $374,999 per year in the US (Zip

Recruiter).


Physical therapists:

Physical therapists often provide helpful strategies and exercises to assist cancer

survivors who have gone through intensive surgeries and or chemotherapy so their physical

function and strength can be returned and that they can return to their daily normal life (Duke

Health). Becoming a physical therapist often takes 6-8 years of higher education. You must first

use four years to acquire a bachelor degree, followed by 2-3 years for a Doctor of Physical

Therapy (DPT) degree. Physical therapists often make $95k per year in the US (Career

Explorer).


Treatments

Introduction to Treatments for Ependymoma

Gliomas:

Ependymomas are a form of glioma, which is a form of brain tumor formed by glial cells

that are within the brain (“Glioblastoma and Other Gliomas > Fact Sheets”). Gliomas are first

formed in the ependymal cells, which are cells that surround fluid-filled areas of the brain and

spinal cord that help form cerebrospinal fluid. Gliomas can either be low or high grade, which is

determined by a biopsy through discovering the abnormalities of the glial cells. Gliomas are

typically found in the lower back portion of the brain in children. Most gliomas relating to

Ependymoma are low grade, meaning they slowly grow and spread in the brain and spinal cord

region (“Definition of ependymoma - NCI Dictionary of Cancer Terms - NCI”).


Exploring Forms of Treatment:

Since Ependymoma is a rare form of brain and spinal cord cancer with cancerous tumors,

this alone presents many significant challenges to the patient and treatment. Treatment is heavily

dependent on the child, and the goal is to create a positive prognosis, educated guess for the

predictions of recovery, survival, and recurrence, which currently has a 50% poor prognosis

percentage with many pediatric patients. The treatments of Ependymoma rely on the uniquenessof the tumor location, size, and grade. There are constant clinical trials and research teams to

help combat the progress of the disease and form a more standardized treatment approach

(Ritzmann et al.).


Early Detection in Ependymoma


Challenges:

There are many challenges associated with the early detection process of Ependymoma.

This can be related to the vague symptoms such as headaches, nausea, vomiting, dizziness, etc.

which make it difficult to identify the origins of these symptoms. Additionally, the location of the

tumor affects the symptoms that someone can experience, leading to further confusion in

symptoms and masking Ependymoma as another condition. Furthermore, Ependymoma is

difficult to diagnose in children as standard growth can mask signs of Ependymoma such as

increased head size from the tumor.


Genetic and Molecular Biomarkers:

There are 3 notable categories that serve as biomarkers of Ependymoma with their

designated markers. There are Supratentorial Ependymomas: ZFTA, RELA, YAP1, MAML2,

Posterior Fossa Ependymomas: H3 K27me3, EZHIP (methylome), and Spinal Cord

Ependymomas: MF2, MYCN (Ghiaseddin).


Immunotherapy

Immunotherapy is a form of cancer treatment where the patient's own immune system is fighting

off the cancerous cells in the body. This is possible by strengthening the immune system through

identifying any weaknesses or blind spots that the immune system has towards cancerous cells

(“What Is Immunotherapy for Cancer?”).


HER2 CAR T cells:

In an undergoing clinical trial conducted by the Pediatric Brain Tumor Consortium, the

organization is experimenting with a form of immunotherapy called HER2 CAR T cells, which

stands for HER2 chimeric antigen receptor T cells. The purpose of the utilization of HER2 CAR

T cells is to target recurrent and progressive tumors. These special cells are formulated from apatient’s blood and insert the HER2 CAR gene into the patient’s T cells. By doing so, it allows

the body to recognize a unique protein located on the tumor; HER2. Any tumors that express the

HER2 protein will be targeted by the patient’s blood (Pediatric Brain Tumor Consortium).


Brain Tumor Antigen Targeted Vaccines:

While investigating treatment methods specifically for Ependymoma, there are also

forms of immunotherapy being used more generally for brain tumors. A common form of brain

tumor is called a glioma, and the purpose of these vaccinations is to identify proteins on the

tumor for the immune system to combat. There are 3 major proteins that these vaccines are

targeting; IL-13Rα2, EphA2, and survivin, all unique to gliomas and differing from healthy brain

cells. This is used to help develop the vaccine as the immune system is given a cytotoxic

T-lymphocyte (CTL) epitope. This vaccine is most compatible with the immune type called

HLA-A2+, which is inhibited by 40-45% of the population. With further research, the future for

this HLA-A2-restricted brain tumor vaccine will be utilized for future Ependymoma treatments

(Pollack).


Therapeutic Interventions

Radiation Therapy:

Ependymoma is commonly treated with a form of therapy that uses radiation and

powerful energy beams aimed at tumor cells. To generate these energy beams, the energy is

stemmed from X-rays and other forms. During the process of the therapy, the machine for

radiation therapy targets the energy beams towards the tumor and kills the tumorous cells.

Radiation therapy is mainly utilized after surgery if the surgery didn’t work out or utilized to

prevent tumor recurrence. There are many forms of radiation therapy, including proton beam

therapy, conformal radiation therapy, and intensity-modulated radiation (Mayo Clinic).


Chemotherapy:

While not a very common form of treatment, it could be used when ependymoma cancer

cells grow back again, or it is very difficult for the cells to be removed by radiation or surgery.

Chemotherapy is used to try and kill or shrink tumor cells. This process can also make it easy fora cell to be removed by surgery or killed by radiation due to its smaller size after chemotherapy.

(Mayo Clinic)


Targeted Therapy:

This form of treatment offers a promising approach to inhibit tumor growth and survival.

Instead of attacking all rapidly dividing cells (chemotherapy), targeted therapy works by

attacking specific molecules in the cells involved in tumor growth (Mayo Clinic). This precision

enables a form of treatments that are more effective and less harmful to healthy tissue, especially

in developing brains. Notch signaling pathways control how cells grow, divide, and develop, this

pathway becomes overactive with ependymomas causing tumor cells to grow uncontrollably.

Drugs that block the Notch pathway slows down tumor growth and makes the cancer cells more

sensitive to other types of treatments (National Library of Medicine). Targeted therapy is a

treatment approach towards more precise and effective care.


Proton Beam Therapy:

Proton beam therapy is an advanced form of radiation treatment and is a highly

recommended approach for managing pediatric ependymomas. Unlike conventional radiation

therapy, which passes through the tumor and exits the body with a risk of affecting healthy

tissues, proton beam therapy uses charged particles (protons) that use their energy directly within

the tumor site and stop there. A method known as the Bragg Peak allows doctors to deliver high

doses of radiation precisely to the tumor while minimizing exposure to the surrounding healthy

tissue (Oncolink). With this precision, proton beam therapy reduces the risk of long-term side

effects such as cognitive deficits, hormonal imbalances, or even development delays.

Proton beam therapy achieves similar tumor control rates as conventional radiation

therapy, but is much more safer making it a preferred choice. Proton beam therapy is a promising

advancement in the treatment of pediatric ependymomas, especially since preservation of

neurological function and quality of life is a top priority (National Library of Medicine).


Clinical Trials:

One of the newer methods of treating Ependymoma includes using clinical trials. Clinical

trials are research studies where doctors and researchers utilize new forms of treatments andmethods of diagnosis on a select group of people to help develop new medications and deem if the medication is safe to implement in future cases. This is especially for Ependymoma because there are new forms of therapies undergoing test and experimentation to help find approaches to combat the brain and spinal cord tumors. Because of how rare Ependymomas are, doctors are hoping to identify multiple methods on how to combat Ependymoma other than the common uses of radiation and surgery. The doctors and scientists aim to find more chemotherapies, targeted therapies, and immunotherapy drugs (“Ependymoma: Diagnosis and Treatment - NCI”).


Advanced Machinery Interventions

Brain Mapping Technology

This treatment method is a minimally invasive procedure to help plan a larger surgery. It

is used to help identify infected tissue, which treatment methods are best for the patient, and help

locate the location of your standard brain functions so no healthy tissue is impacted during

surgery (“Brain Mapping Technology”).

There is a common form of brain mapping technology utilized called diffusion tensor

imagining with white matter tractography. It uses technology to simulate a 3-D map of the brain.

It helps to “Determine the best surgical approach to remove a tumor, diagnose and treat traumatic

brain injuries, find breaks in brain fibers that may cause the loss of certain body and memory

functions, understand which brain connections are intact and which are damaged” (Brain

Mapping Technology).


Non Pediatric Treatment

Treatment for Adults:

Treatment can depend on where the Ependymoma is located. Patients who are older

(Ages 30-40) are more likely to develop ependymoma in their spinal cord. Treatments for adults

can include surgery, which helps remove the tumor from the spinal cord. For low-grade

ependymoma, so Grade I or Grade II, completely removing the ependymoma is often the goal,

and the Neuro-Oncology team focuses on complete surgical removal to ensure the patient's

recovery (“Treatment for Adults”). If complete surgical removal of the Ependymoma is not

possible, then radiation therapy is utilized, even after partial surgical removal. Spinalependymoma surgery is used for patients with ependymoma in the spinal cord. This procedure involves a surgeon who removes some parts of the bone to have access to the spinal canal with the tumor, and then using a microscope to carefully remove the tumor (Kaiser). During this surgery, anesthesia is given to the patient, and most patients will be able to walk 1-2 days after the operation (Kaiser).


Statistics

Survival Rates

Overview:

The diagnosis rate for Ependymoma are typically very low, as Ependymomas account for

below 2% of brain and central nervous system tumors, and only account for 9% of central

nervous system and brain tumors in children. The survival rates for Ependymoma are generally

high despite a low prognosis percentage. When observing the rates for non-malignant versus

malignant, the non-malignant 5 year relative survival rates statistics are generally higher than

malignant. Regarding non-malignant in children 0-14, there is a 96.8% survival rate and

malignant have a 74.7% survival rate. Regarding non-malignant in adolescents and young adults

15-19, there is a 98.7% survival rate and malignant have a 91.7% survival rate. Finally, regarding

non-malignant in adults, there is a 95.8% survival rate and malignant have an 86.4% survival rate

(Ghiaseddin).


Rate by Grade:

Although there are varying grades of Ependymoma determining how aggressive the

tumor is, it is important to consider that location has a great determination in survival. Tumors

found in the spine typically have higher survival rates than tumors found intracranially, a

constant pattern seen between the 3 grades (Villano et al.).


Grade I Ependymoma, also known as Subependymoma, is a very slow progressing tumor

and is benign. The 5 year survival rate is very high with about >90% survival rate (Villano et al.).


Grade II Ependymoma, also known as Classic Ependymoma, is faster progressing than

Grade I and is the most common type. They have a higher recurrence rate than Grade I. The 5

year survival rate is moderately high with about 70-85% survival rate (Villano et al.).


Grade III Ependymoma, also known as Anaplastic Ependymoma, is the most aggressive

tumor. Children are most likely to be Grade III if they have a malignant tumor. The 5 year

survival rate is moderate with about 40-60% survival rate (Villano et al.).


Demographic Statistics

Gender:

Males typically have a higher diagnostic rate than females do. While there isn’t a

biological reason as to why the tumor occurs more in males, there is a lower overall survival rate

in males, with males having a 71% and females having a 78% (Mestnik et al.).


Race and Ethnicity:

White patients have the highest percentage of Ependymoma cases, accounting for about

87% of all cases. The rate is about 0.44 per 100,000 (Villano et al.).


Black patients have the second highest percentage of Ependymoma cases, accounting for

about 7.6% of all cases. The rate is about 0.25 per 100,000 (Villano et al.).


Asian/Pacific islander have the third highest percentage of Ependymoma cases,

accounting for about 2.4% of all cases. The rate is about 0.26 per 100,000 (Villano et al.).


American Indian/Alaska Native have the lowest percentage of Ependymoma cases,

accounting for about 0.7% of all cases. The rate is about 0.20 per 100,000 (Villano et al.).


Regarding ethnicity, Non-Hispanic patients have the highest percentage of total cases,

with an 88.4% and a rate of 0.43 per 100,000. Hispanic patients account for 11.6% of total cases,

with a rate of 0.34 per 100,000 (Villano et al.).


Tumor Localization

While there isn’t specific data regarding survival rates for locations and age and gender, there are

certain tumor locations with certain prognosis with their respective molecular genetic markers

and immunohistochemical markers (Zaytseva et al.).


ST-EPN-ZFTA:

  • Location: Supratentorial

  • Age: All ages, mainly children

  • Molecular genetic markers: ZFTA fusions, chromothripsis 11q13.1

  • Immunohistochemical markers: p65 (RelA); L1CAM; Cyclin D1

  • Prognosis: Poor


ST-EPN-YAP1

  • Location: Supratentorial

  • Age: Young children, mainly females

  • Molecular genetic markers: YAP1 fusions

  • Immunohistochemical markers: N/A

  • Prognosis: Favorable


PF-EPN-A

  • Location: Infratentorial

  • Age: Young children, mainly males

  • Molecular genetic markers: CpG-island methylator phenotype

  • Immunohistochemical markers: H3K27me3; EZHIP overexpression

  • Prognosis: Poor

PF-EPN-B:

  • Location: Infratentorial

  • Age: Adolescents and young adults

  • Molecular genetic markers: Major cytogenetic aberrations

  • Immunohistochemical markers: H3K27me3●

  • Prognosis: Favorable


Charts, Tables, and Data

ree

This chart demonstrates how over the years

after an Ependymoma diagnosis, the survival

rates start to decrease. This chart

demonstrates resection in pediatric patients of

Grades I, II, and III Ependymoma (Marinoff,

A.E., Ma, C., Guo, D. et al.).


ree

This chart is more specifically highlighting a

comparison between Grades II and III. We

can clearly see that Grade III has a

significantly low overall survival rate due to

the aggressiveness of the grade. One aspect of

the graph that stands out is the vertical line at

around 21 years post-diagnosis, which aids in

demonstrating how the prognosis for Grade

III does not have a good long term outlook

(Marinoff, A.E., Ma, C., Guo, D. et al.).


Impacts

Overview:

Experts are still unaware as to why the ependymal cells in the brain become cancerous

and develop into ependymoma tumors. This shows a severe gap in information and withholds

important information in treatment of ependymomas. Ependymomas account for nearly 5-10% of

pediatric tumors, coming out to be almost 200 cases every year (Nationwide Children’s

Hospital). Ependymomas are the most commonly found tumor in children under five years of

age. The impacts of ependymomas can be categorized into 4 main impacts, being (1)

Neurological and Cognitive Impacts, (2) Emotional and psychological effects, (3) Physical and

Developmental Challenges and (4) Academic and Social challenges.


Neurological and Cognitive Impacts:

Due to the tricky location of ependymomas being found on the posterior fossa or on the

spinal cord, ependymoma patients can experience increased intracranial pressure, cranial nerve

deficits (referring to the loss in functioning in one or more cranial nerve), headaches, nausea, as

well as body-balance issues (Packer, R. J., & Vezina, G, 2008). Treatments to remove and

eradicate ependymomas like surgical intervention or radiation therapy may lead to long term

neurocognitive effects like memory loss, learning difficulties and deficits in attention (Merchant

et al, 2009).


Emotional and psychological effects:

Pediatric Ependymoma patients can experience anxiety, depression and emotional

distress before and after treatment which can be due to both (1) neurological side effects from the

ependymoma itself, and (2) the age of the patient (Packer, R. J., & Vezina, G, 2008). Intracranial

pressure and balance issues as neuro-cognitive side effects from the positioning of the

ependymoma may cause psychological strain, especially in pediatric patients who are still

understanding their bodies. These effects can persist and are reported in survivors of

ependymomas (Merchant et al., 2009).


Physical and Developmental Challenges:

Treatment related physical complications, especially craniospinal irradiation, can lead to

growth retardation, fatigue and endocrine dysfunction. Physical developmental delays and the

need for long-term rehabilitation techniques are common outcomes as well (Packer, R. J., &

Vezina, G, 2008).


Academic and Social Challenges:

Due to frequent hospitalizations, fatigue and neuro-cognitive effects, many pediatric

ependymoma patients have trouble returning to school, experience academic underperformance

and even social isolation (Merchant et al., 2009).


Tumor Biology and Recurrence Risk:

An important possible impact of ependymomas is the recurrence risk of tumors. Recent

advancements in molecular classification revealed that different molecular subtypes of

ependymomas have variable outcomes, i.e. different subtypes have different risks of recurrence,

with some posing higher risks than others (Pajtler et al., 2015). For example, Posterior Fossa

group A ependymoma tumors (PFA), have a poorer prognosis and higher recurrence rate due to

their CIMP-Negative molecular structure (Witt et al). Recurrence is more likely in cases where

full tumor resection is not achieved, especially for tumors located in surgically-challenging areas

like the posterior fossa. A prospective group found through research that even conformal

radiotherapy following surgery still keeps the recurrence rate highly concerning in long-term

outcomes (Merchant et al). In the event of recurrence, treatment options become limited and

tumors begin to show resistance towards traditional therapies. Common approaches to eradicate

tumor-recurrence include repeat surgery, re-irradiation and experimental therapies (Fukuoka et

al).


Conclusion

Ependymomas continue to remain a challenging yet critical area in pediatric oncological

research due to their complex behaviour, diverse anatomical positions and locations, and varied

responses towards treatment. This paper explored and reviewed the current understanding of

ependymomas including their diagnostics and classification, to treatment strategies such as

surgical intervention, radiation therapy, and the proton beam therapy. Despite advancements,

many patients often face long-term consequences that have significant neurological, cognitive,

developmental and even emotional and psychological impacts; particularly when the tumor is

found to be situated in a particularly sensitive region of the brain. Continued research is essential

to refine diagnostic techniques and treatment plans, as well as improve outcomes. A deeper

understanding of molecular and genetic foundation under the cause and occurrence of

ependymomas may pave the way for targeted therapies, while better psychosocial support can

help address the extensive emotional toll of the disease. In conclusion, improving both survival

and quality of life in children impacted by ependymomas must remain a central focus for

research and advancements in the future.


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