Generality
Ependymoma is a brain tumor that originates in ependymal cells; these cells line the ventricles of the brain and the central canal of the spinal cord.
The diagnostic process includes several tests, as before the therapy it is useful to trace the exact location and severity of the tumor.
Ependymomas that lend themselves to surgical removal should be removed. Subsequent treatments (radiotherapy and chemotherapy) depend on the quality of the removal procedure.
Brief reminder of brain tumors
When we talk about brain tumors, or brain tumors or brain neoplasms, we are referring to benign or malignant masses of cancer cells affecting the brain (hence an area between the telencephalon, diencephalon, cerebellum and brain stem) or the spinal cord . Together, the brain and spinal cord form the central nervous system (CNS).
The result of genetic mutations, the exact cause of which is not known very often, brain tumors can:
- originate directly from a cell of the central nervous system (in this case we also speak of primary brain tumors);
- derive from a malignant tumor present in other locations in the body, such as the lungs (in this second case they are also called secondary brain tumors).
Given the extreme complexity of the central nervous system and the large number of different cells that compose it, there are many different types of brain tumors: according to the latest estimates, between 120 and 130.
Regardless of their malignancy or not, brain tumors must almost always be removed and / or treated with radiotherapy and / or chemotherapy, as they often cause neurological problems incompatible with a normal life.
What is ependymoma?
Ependymoma is a brain tumor that originates from the ependyma, or the epithelium that lines the cerebral ventricles and the central canal of the spinal cord.
Ependyma is so called because it is made up of particular cells of the glia, known as ependymocytes or more simply ependymal cells.
Ependymomas can be both neoplasms of a benign nature and neoplasms of a malignant nature.
Difference between a benign tumor and a malignant tumor
A benign tumor is a mass of abnormal cells that grows slowly, has little infiltrative power and an "equally poor (if any) metastasizing power."
On the contrary, a malignant tumor is an abnormal cell mass that grows quickly, has a high infiltrative power and almost always a high metastasizing power.
NB: by infiltrative power, s "means the ability to affect adjacent anatomical regions. With metastatic power, on the other hand, we refer to the ability of tumor cells to spread, through the blood or the lymphatic circulation, to other organs and tissues of the body (metastasis).
GLIA, GLIA CELLS AND EPENDYM
With its cells, the glia provides support, stability and nourishment to the intricate network of neurons, present inside the human body and having the task of transmitting nerve signals.
Brain ventricles (highlighted in gold). In the central nervous system, the cellular elements of glia are astrocytes, oligodendrocytes, ependymal cells and microglia cells.
In the peripheral nervous system (PNS), the cellular elements of the glia are Schwann cells and satellite cells.
Thanks to the activity of the ependymocytes, the ependyma is involved in the circulation and production of the cerebrospinal fluid (or liquor).
TYPICAL LOCATION OF EPENDYMOMES
Ependymomas can develop in both the brain and the spinal cord.
Those with brain origin may occasionally spread to the spinal cord via the CSF.
EPENDIMOMA: TYPES AND THEIR DEGREE OF GROWTH
Central nervous system (CNS).
Brain tumors are divided into 4 degrees - identified with the first four Roman numerals - according to their growth power.
Grade I and II brain tumors grow very slowly and involve a "small area of the brain; they are usually benign."
Conversely, grade III and IV brain neoplasms rapidly expand and invade surrounding tissue regions; they are generally malignant in nature.
A grade I or II brain tumor can, over time, turn into a grade III or IV tumor.
There are at least four types of ependymoma, differing in characteristics and, in 3 out of 4 cases, also in degree:
- The subependymoma. It is a very slow growing grade I brain tumor, which usually forms near the ventricles of the brain
- Myxopapillary ependymoma. Like subependymoma, it is a slow-growing Grade I brain tumor, however, unlike the previous case, it tends to develop in the lower parts of the spinal cord.
- Ependymoma proper. It is a slow-growing Grade II glioma that can originate in or in close proximity to the ventricles of the brain.
- The anaplastic ependymoma. It is a grade III malignant brain tumor, which usually appears in the brain (near the ventricles) or in the posterior cranial fossa and only in a few cases in the spinal cord. Like any malignant tumor, it has a growth rate very quick.
EPIDEMIOLOGY
Ependymomas account for 2-4% of all central nervous system cancers.
They can affect both adults and children: in the former, they are quite rare and mainly affect individuals under the age of 45; in the second, they constitute the sixth most common brain tumor and particularly affect individuals under 3 years of age (30% of cases).
Causes
Ependymomas, as well as almost all human brain tumors, arise for reasons that are not yet known.
RISK FACTORS
Doctors and researchers agree that there are at least two situations that can favor the appearance of an ependymoma:
- Previous radiation treatment to the head. It is necessary to specify that some scholars have, in this regard, a "different opinion: they believe, in fact, that radiotherapy has no harmful effect.
- Suffering from type II neurofibromatosis, a rare genetic-hereditary disease that causes the appearance of various tumors in the nervous system.
Symptoms and Complications
The symptoms and signs of an ependymoma depend on the site of onset of the ependymoma.
If the tumor resides in the spinal cord, the patient usually feels:
- Pain in the neck or back, depending on the precise location of the tumor mass.
- Numbness and / or weakness in the arms or legs.
- Problems with bladder control.
If the neoplasm is located in the brain, the symptom picture usually consists of:
- Headache
- Nausea and vomiting, especially in the morning
- Seizures
- Poor eyesight. They arise if the ependymoma resides near the optic nerve.
- Numbness and a sense of weakness in the limbs (both upper and lower) of only one side of the body. They are typical disorders when ependymomas affect the frontal or parietal lobe of the brain.
- Problems with coordination and balance. They are typical symptoms of when the ependymoma has taken place close to the temporal lobe of the brain.
- Changes in mood (eg sudden irritability) and personality changes. They occur when the ependymoma is near the frontal lobe of the brain.
HEADACHE, NAUSEA AND VOMITING
Headache, nausea and vomiting are due to an increase in intracranial (or intracranial) pressure. This increase can occur for two reasons, often concomitant:
- Because the growing tumor mass opposes the normal flow of cerebrospinal fluid.
- Because edema forms around the tumor mass
If severe, the alteration of the normal flow of CSF can lead to the onset of a pathological condition known as hydrocephalus.
RHYTHM OF APPEARANCE OF SYMPTOMS
Symptoms of a Grade I or II ependymoma appear gradually (it may take months), as the tumor mass has a slow growth rate.
Quite the opposite, the symptoms of a grade III ependymoma tend to occur shortly after the appearance of the neoplasm, as the rate at which the tumor mass grows is very rapid.
Diagnosis
When faced with a suspected case of ependymoma, doctors begin their diagnostic investigations with a thorough physical examination and "analysis of tendon reflexes."
Then, they perform an eye test and ask the patient some questions aimed at assessing the mental state and cognitive abilities (reasoning, memory, etc.).
Finally, to dispel any doubts and to know the exact location and size of the tumor, they resort to specific tests such as:
- Nuclear magnetic resonance
- CT scan (or computed axial tomography)
- Biopsy of the tumor
- Lumbar puncture
OBJECTIVE AND TENDON REFLEX EXAMINATION, EYE TEST AND MENTAL-COGNITIVE EVALUATION
- The physical examination involves the analysis of symptoms and signs, reported or manifested by the patient. Although it does not provide any certain data, it can be very useful for understanding the type of pathology in progress.
- By examining the tendon reflexes, the doctor assesses the presence or absence of neuromuscular and coordinative disorders.
- Using an eye test, the doctor observes the optic nerve and analyzes its involvement.
- The assessment of mental status and cognitive abilities is carried out with the aim of understanding which area of the central nervous system may have developed a neoplasm. For example, finding disorders in the lower limbs alone suggests a neurological problem based in the spinal cord rather than in the brain and so on.
NUCLEAR MAGNETIC RESONANCE (NMR)
The nuclear magnetic resonance (NMR) is a painless diagnostic test, which allows to visualize the internal structures of the human body without the use of ionizing radiation (X-rays).
Its operating principle is quite complex and is based on the creation of magnetic fields, which emit signals capable of being transformed into images by a detector.
The magnetic resonance of the brain and the medulla provide a satisfactory view of these two compartments. However, in some cases, to optimize the quality of the visualization, it may be necessary to inject a venous contrast liquid into the vein. In such situations, the test becomes minimally invasive, because the contrast fluid (or medium) could have side effects.
A classic MRI takes about 30-40 minutes.
CT scan
CT is a diagnostic procedure that uses ionizing radiation to create a highly detailed "three-dimensional" image of the body's internal organs.
Although it is painless, it is considered invasive due to X-ray exposure. Also, like MRI, it may require the use of a contrast agent - not free from possible side effects - in order to improve the quality of the visualization.
A classic CT scan takes about 30-40 minutes.
BIOPSY
A tumor biopsy consists in taking and histological analysis, in the laboratory, of a sample of cells coming from the neoplastic mass. It is the most suitable examination if you want to trace the main characteristics of a tumor (type, grade and malignancy).
The removal of cells from an ependymoma usually takes place under general anesthesia (therefore with the patient asleep) and involves the puncture of the skull, in order to insert a special needle for sampling in the desired area.
Obviously, the tumor biopsy takes place after identifying the precise site of the neoplasm.
LUMBAR PUNCTURE
Lumbar puncture consists of taking the cerebrospinal fluid and analyzing it in the laboratory.
To withdraw the CSF, a needle is used that the doctor inserts between the lumbar vertebrae L3-L4 or L4-L5. A local anesthetic injection is obviously made at the insertion point.
Performing a lumbar puncture in the presence of an ependymoma serves to determine whether the tumor has spread from the brain to the CSF.
Treatment
When the ependymoma does not reside in an inaccessible location, it is good to do everything possible to remove it completely or to a large extent.
The use of further treatments - in this case radiotherapy and, sometimes, even chemotherapy - depends on the "extent of the removal" and on the characteristics of the tumor.
SURGERY
The ependymomas that lend themselves to complete removal are those of grade I or II, small in size and located in an easy-to-reach position.
On the contrary, the ependymomas that can only be partially removed are those of grade III and those of grade I or II located in uncomfortable and difficult to reach locations.
At the end of the removal operation (whether partial or total, it does not matter), the patient is required to observe a phase of complete rest, followed by a period of physiotherapy.
Advantages of total removal
In addition to making radiotherapy treatment superfluous, the total removal of the tumor mass can also mean total recovery from the neoplasm.
RADIOTHERAPY
Tumor radiotherapy is the treatment method based on the use of high-energy ionizing radiation, with the aim of destroying neoplastic cells.
It is adopted in case of ependymoma when:
- The surgical removal operation is impractical. This occurs when the tumor mass is in a position that cannot be reached by the surgeon.
- The surgical removal of a grade I or II tumor was partial, so there are cancer cells left.
- The neoplasm was grade III. In these situations, the residual neoplastic cells have a strong tendency to reform the ependymoma again (relapse) and to affect the healthy brain mass.
CHEMOTHERAPY
Chemotherapy is the administration of drugs capable of killing all rapidly growing cells, including cancer cells.
It is taken into account when there is a relapse.
SYMPTOMATIC TREATMENT
To prevent seizures and relieve disorders associated with tumor edema, doctors may prescribe anticonvulsants and corticosteroids, respectively.
Main side effects of radiotherapy
Main side effects of chemotherapy
Main side effects of corticosteroids
Tiredness
Itching
Hair loss
Nausea
He retched
Hair loss
Sense of fatigue
Infection vulnerability
Osteoporosis
Obesity
Indigestion
Hypertension
Agitation
Sleep disorders
Prognosis
The prognosis of an ependymoma improves if:
- The tumor is low-grade (better grade I than grade II).
- Diagnosis is timely. This is especially true for grade III malignant ependymomas.
- The tumor mass occupies a comfortable position and is small in size. A large, non-removable ependymoma can be fatal even if it is grade I or II.