Generality
Ependymoma is a brain tumor that originates in ependymal cells ; these cells line the cerebral ventricles and the central canal of the spinal cord.
The diagnostic procedure includes several tests, since before the therapy it is useful to go back to the exact position and the gravity 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 reference to brain tumors
When we talk about brain tumors, or brain tumors or brain neoplasms, we refer to benign or malignant masses of tumor cells that affect the brain (ie an area between the telencephalon, diencephalon, cerebellum and brainstem) or the spinal cord . Together, encephalon and spinal cord form the central nervous system ( CNS ).
Fruit of genetic mutations, of which however the precise cause 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 sites of 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 malignant power or not, brain tumors are almost always removed and / or treated with radiotherapy and / or chemotherapy, as they often cause neurological problems incompatible with a normal life.
What is the ependymoma?
The ependymoma is a brain tumor that originates from the ependyma, or the epithelium that covers the cerebral ventricles and the central canal of the spinal cord.
The ependima 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 either malignant neoplasms or malignant neoplasms.
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 not zero) metastasizing power.
Conversely, a malignant tumor is an abnormal cell mass that grows quickly, has high infiltrative power and almost always a high metastasizing power.
NB: due to infiltrative power, this refers to the ability to affect adjacent anatomical regions. With metastasizing power, on the other hand, reference is made to the ability of cancer cells to spread, through blood or lymphatic circulation, to other organs and tissues of the body (metastasis).
GLIA, CELLS OF THE GLIA AND EPENDIMA
With its cells, the glia provides support, stability and nourishment to the intricate network of neurons, present within 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 the glia are astrocytes, oligodendrocytes, ependymal cells and microglia cells.
In the peripheral nervous system ( SNP ), the cellular elements of the glia are Schwann cells and satellite cells.
Thanks to the activity of ependymocytes, ependyma is involved in the circulation and production of the cerebrospinal fluid (or CSF ).
TYPICAL LOCALIZATION OF EPENDIMOMES
Ependymomas can develop both in the brain and in the spinal cord.
Those with encephalic origin may occasionally spread in the spinal cord through the liquor.
EPENDIMOMA: TYPOLOGIES AND THEIR DEGREE OF GROWTH
Central nervous system (CNS).
Brain tumors are divided into 4 degrees - identified with the first four Roman numerals - depending on their growth power.
Grade I and II brain tumors grow very slowly and involve a restricted brain area; generally they are benign.
In contrast, grade III and IV brain tumors expand with speed and invade the surrounding tissue regions; they are generally malignant.
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 ependymomas, different in characteristics and, in 3 cases out of 4, also in degree:
- The subependimoma . It is a very slow-growing brain tumor of grade I, which usually forms near the cerebral ventricles
- The ependymoma mixopapillates . Like the subependimoma, it is a slow-growing brain tumor of grade I; however, unlike the previous case, it tends to develop in the lower parts of the spinal cord.
- The true ependymoma . It is a slow-growing grade II glioma, which can originate within or in close proximity to the cerebral ventricles.
- Anaplastic ependymoma . It is a malignant brain tumor of grade III, 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 very rapid growth rate.
Epidemiology
Ependymomas represent 2-4% of all central nervous system tumors.
They can affect both adults and children: in the former, they are quite rare and mostly affect individuals under the age of 45; in the latter, they constitute the sixth most common brain tumor and particularly concern individuals under 3 years (30% of cases).
Causes
Ependymomas, like almost all human brain tumors, arise for reasons that are not yet known.
RISK FACTORS
Doctors and researchers agree that at least two situations exist that can favor the appearance of an ependymoma:
- A previous radiotherapy treatment for the head . It should be noted that some scholars have a different opinion on this subject: 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 several tumors in the nervous system.
Symptoms and Complications
The symptoms and signs of an ependymoma depend on the site of onset of the ependymoma itself.
If the tumor resides in the spinal cord, the patient generally feels:
- Neck or back pain, 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 symptomatic 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 weakness in the limbs (both upper and lower) of one side of the body. They are typical disorders when ependymomas affect the frontal or parietal lobe of the brain.
- Coordination and balance problems. They are typical symptoms of when the ependymoma has taken place behind the temporal lobe of the brain.
- Mood changes (for example 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 the cerebrospinal fluid.
- Because edema forms around the tumor mass
SYMPTOMS APPEARANCE RHYTHM
The 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 contrary, the symptoms of a grade III ependymoma tend to occur shortly after the appearance of the tumor, as the rate at which the tumor mass increases is very rapid.
Diagnosis
Faced with a suspected ependymoma case, doctors begin their diagnostic investigations by a careful physical examination and an analysis of tendon reflexes .
Then they perform an ocular test and ask the patient some questions aimed at assessing mental status and cognitive abilities (reasoning, memory, etc.).
Finally, to dispel any doubts and to know the position and exact size of the tumor, they resort to specific tests such as:
- Nuclear magnetic resonance
- CT scan (or computerized axial tomography)
- Tumor biopsy
- Lumbar puncture
EXAMINATION OBJECTIVE AND TENDONS TRIMS, 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 place.
- With the examination of tendon reflexes, the doctor evaluates the presence or absence of neuromuscular and coordinative disorders.
- Through an ocular test, the doctor observes the optic nerve and analyzes its involvement.
- The assessment of mental status and cognitive abilities is carried out with the intent to understand which area of the central nervous system may have developed a neoplasm. For example, finding lower limb alone disorders suggests a neurological problem located in the spinal cord, rather than in the brain and so on.
NUCLEAR MAGNETIC RESONANCE (RMN)
Nuclear magnetic resonance ( NMR ) is a painless diagnostic test that allows the visualization of 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 encephalon and the medulla provide a satisfactory view of these two compartments. However, in some cases, to optimize the quality of the display, it may be necessary to inject a contrast liquid to a venous level. In such situations, the test becomes minimally invasive, because the contrast liquid (or medium) could have side effects.
A classic MRI lasts about 30-40 minutes.
TAC
CT scan is a diagnostic procedure that uses ionizing radiation to create a highly detailed three-dimensional image of the internal organs of the body.
Although it is painless, it is considered invasive due to exposure to X-rays. Furthermore, like MRI, it may require the use of a contrast agent - not without possible side effects - in order to improve the quality of the display.
A classic CT scan takes about 30-40 minutes.
BIOPSY
A tumor biopsy consists of the collection and histological analysis, in the laboratory, of a sample of cells from the neoplastic mass. It is the most suitable test if you want to go back to the main characteristics of a tumor (type, grade and malignancy).
The collection of cells from an ependymoma takes place, usually, under general anesthesia (therefore with the patient asleep) and involves the drilling of the skull, in order to insert a special needle for the collection in the desired area.
Obviously, tumor biopsy occurs after having identified the precise site of the neoplasm.
LUMBAR PUNCTURE
The lumbar puncture consists in the collection of the cerebrospinal fluid and in its analysis in the laboratory.
To remove the liquor, a needle is used that the doctor inserts between the lumbar vertebrae L3-L4 or L4-L5. At the insertion point, an injection of local anesthetic is obviously practiced.
The execution of a lumbar puncture in the presence of an ependymoma is used to establish whether the tumor has spread from the encephalon to the liquor.
Treatment
When the ependymoma does not reside in an inaccessible position, 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 degree I or II, of small dimensions 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 complete rest phase, followed by a period of physiotherapy .
Benefits of total removal
In addition to making radiotherapy treatment superfluous, 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 the neoplastic cells.
It is adopted in case of ependymoma when:
- The surgical removal operation is impractical . This happens when the tumor mass is in a position unattainable by the surgeon.
- Surgical removal of a grade I or II tumor was partial, therefore tumor cells remained.
- The neoplasm was grade III . In these situations, the residual neoplastic cells have a strong tendency to reform the ependymoma (recurrence) and to affect the healthy brain mass.
CHEMOTHERAPY
Chemotherapy consists of the administration of drugs capable of killing all rapidly growing cells, including cancer ones.
It is taken into consideration when there is a recurrence.
SYMPTOMATIC TREATMENTS
To prevent seizures and to alleviate disorders related to tumor edema, doctors could prescribe anticonvulsants and corticosteroids, respectively.
Main side effects of radiotherapy | Main side effects of chemotherapy | Major side effects of corticosteroids |
Fatigue itch Hair loss | Nausea He retched Hair loss Sense of fatigue Vulnerability to infections | Osteoporosis Obesity Indigestion Hypertension agitation Sleep disorders |
Prognosis
The prognosis of an ependymoma improves if:
- The tumor is low grade (better than grade I than grade II).
- The 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 and non-removable ependymoma can be lethal even if of grade I or II.
