Primary Brain Tumor

Click on the links below to jump to paragraphs per subject:
Glioma, Ependymoma, Meningioma, Adenoma or Craneopharyngeoma, Angioma, Medulloblastoma, Pineal gland tumor, Brain cyst and Vestibular Schwannoma

Primary brain tumors are tumors that arise from the brain tissue itself and are not a metastasis from a tumor elsewhere in the body.

The complaints vary and depend on the location in the brain and the growth rate and size of the tumor, the type or type of brain tumor.
The brain controls everything in the body. As a result, in addition to the specific complaints of a tumor, all kinds of symptoms can occur in the body.
Frequently heard complaints are:

• Epileptic attacks
  
• Headache
  
• High cerebral pressure
  
• Nausea
  
• Dizziness
  
• Changes in cognitive functioning (thinking, memory problems or slowing down in thinking, difficulty with executive functions, etc.)
  
• Changes in behavior: confusion, psychosis, disinhibited behavior
  
• Changes in feelings/emotions: depression, anger, mood.
  
• Symptoms of failure These can be half-sided complaints such as half-sided loss of strength or half-sided paralysis on the left or right.
  
• Change in motor skills
  
• Speech or language disorders
  
• Changes in vision/visual complaints
  
• Changes in smell and taste
  
• Swallowing disorders
  
• Changes in hearing
  
• Changes in the hormones

Different forms of brain tumors

Tumor of the brain tissue (Glioma)

In many adults, a primary brain tumor is often (15-20%) a glioma, arising from glia cells, the support cells of the brain tissue.
Gliomas are divided into degrees of malignancy I-IV. Low grade I and II, slow growing and III and IV high grade and malignant.
The more tumor cells found per square centimeter, the higher the grade.

Furthermore, gliomas are subdivided based on the type of glia cells from which they arise.

The WHO (World Health Organization) has established a classification of tumors of the central nervous system which is used worldwide since 2016. More information about this classification can be found here. 

The main types of gliomas 

• astrocytomas that arise from star-shaped cells (astrocytes (support cells)). These tumors are the most common.
  • Grade I: pilocytic astrocytoma (juvenile pilocytic astrocytoma)
  • Grade I: cerebellar astrocytoma
  • Grade I: desmoplastic infantile astrocytoma
  • Grade II: diffuse astrocytoma (low-grade or grade 2 astrocytoma)
    • fibrillary
    • mistocytic
    • protoplasmic astrocytoma
  • Grade III: anaplastic astrocytoma
  • Grade IV: gliobastoma multiforma (astrocytoma class IV or GBM 4)
    • astrocytoma class IV-primary
    • novo and secondary
  • Grade V: Subependymal giant cell astrocytoma
  • oligodendrogliomas arise from oligodendrocytes, the insulating layer cells
  • Brain stem glioma
  • ependymomas arise from ependymal cells that line the inner wall of the brain cavities/ventricles through which the cerebrospinal fluid flows. These tumors are quite rare.

Ependymoma

An ependymoma is a brain tumor that arises in the ependymal cells. These brain cells line the inside of the brain cavities (ventricles) and the central canal of the spinal cord. As a result, ependymomas also occur in the spinal cord. The cause may be an error in the DNA of the cells that form the lining of the brain cavities.
It can arise in the cerebrum, the brain
small brain or the spinal canal.
Complaints therefore depend on the location of the tumor and can manifest themselves in:

• headache
• pain elsewhere
• dizziness
• nausea or vomiting
• epileptic attacks
• looking cross-eyed
• swallowing or speech problems
• loss of strength in arms or legs or in the face
• sensory disturbances in arms or legs
• problems walking or urinating
  

This form of tumor may spread via the cerebrospinal fluid and thus may cause metastases.

More information can be found here.

Tumor in the meninges (Meningioma)

A tumor in the meninges, a meningioma, can occur anywhere where the meninges are located:
- around the brain and spinal cord
- between the brain where it forms a septum
- at the bottom of the skull
A tumor in the meninges is a slow-growing and 'relatively benign' tumor; the symptoms arise just as slowly.

Because these tumors are sensitive to hormones, meningiomas are more common in women than in men.

There is also a rare malignant form of this tumor in which 5-7% of it tends to grow into the brain and to return in several places in the meninges.
In 1-2% there is a truly malignant form that spreads. A meningioma can compress brain tissue and cause damage and cause complaints, but these tumors do not grow into the brain. The symptoms caused by a meningioma depend on the location of the tumor.
The complaints may include loss of function and behavioral changes.

Tumor in the pituitary gland (Adenoma or craniopharyngeoma)

A craniopharygeoma is a rare and benign tumor. Benign in the sense that it does not spread (metastasize) to other parts of the body.
During development into a pituitary gland, cells can remain behind and develop into a tumor.
The symptoms of a craniopharygeoma depend on whether the tumor is located inside (sellar) or above (suprasellar) the 'Turkish saddle'. The Turkish saddle is an anatomical structure located at the base of the skull.

A pituitary tumor can also be located in the sphenoid cavity (sphenoidal) or in the nasopharyngeal cavity (nasopharyngeal).

It is also important for the symptom whether the tumor, for example, presses against the hypothalamus or optic nerves or pituitary gland itself.

Symptoms of a tumor in the pituitary gland or craniopharyngioma may include:

• headache
• blurred vision
• double vision
• sometimes visual impairment or blindness (pressure on the optic nerves)
• a deficiency of hormones
• fatigue
• poor recovery from fatigue
• joint problems
• delayed puberty
• problems with weight
• eating disorders
• problems with fat metabolism
• problems with heart function

Pituitary tumors can also be the cause of excessive growth of body extremities and organs (acromegaly) and of a life-threatening deficiency of the stress hormone cortisol (Addisonian crisis).
Read more.
Tumors in the area surrounding the pituitary gland can also press on the pituitary gland itself and thus disrupt its function. Since the optic nerve pathways (optic chiasm) close to the pituitary gland, pressure on those nerve pathways can cause visual complaints such as hemianopia (visual field loss).
Above the tumor, the cerebral cavities may be enlarged because the cerebrospinal fluid is difficult to drain.

An operation (a transsphenoidal neurosurgical procedure) for a pituitary disorder can also damage one of the cranial nerves that control the eye muscles. For example, in the case of a Pituitary NeuroEndocrine Tumor (PitNET). A gaze paralysis may arise after the operation. This means that there is then an inability to look at a certain direction with both eyes.
In case the tumor presses on the hypothalamus, this can cause hypothalamic syndrome. Depending on which area in the hypothalamus is affected, it can have consequences such as eating disorders, sleep disorders, problems with fluid balance, temperature regulation and sexual functions.

The image below explained:
The pink part in the picture is the pituitary gland.

Light pink: the anterior pituitary gland

Dark pink: the posterior pituitary gland

https://commons.wikimedia.org/wiki/File:Grays_pituitary.png

Tumor in the blood vessels (Angioma)

A tumor in the cerebral blood vessels is usually benign. It is a red to purple blood vessel tumor that is caused by dilation and/or a twisting of blood vessels, for example a tangle of varicose veins.

- strawberry hemangioma (or infantile hemangioma)
- cavernous hemangioma
- lymphangioma
- star-shaped hemangioma
- wine stain

Medulloblastoma (PNET)

The medulloblastoma tumor is located, as the name indicates, in the medulla oblongata, a part of the brainstem. But usually in the fourth cerebral cavity (ventricle).
A blastoma is a precursor of brain cells. These cells should have developed into brain cells. Therefore, this tumor mainly occurs in children.
Only 10-20 times a year the diagnosis is made in adults in the Netherlands. A medulloblastoma is a malignant brain tumor.
A medulloblastoma is a tumor that is in the group of PNET tumors, an abbreviation for Primitive Neuro-Ectodermal Tumor.

• Primitive: consisting of precursor cells in a phase in which an unborn child is formed.
  
• Neuro = primitive cells that should have developed into brain cells, but for some reason did not.
  
• Ectodermal = precursor cells of ectoderm tissue. This produces tissues such as the brain and skin.
  
• Tumor

Complaints of medulloblastoma may include:

• Complaints of hydrocephalus/hydrocephalus, because the cerebrospinal fluid cannot drain properly because the tumor is usually located in the fourth cerebral cavity (ventricle). This can cause a headache, which is often accompanied by nausea and vomiting.
  
• Increased pressure in the head which can put pressure on the eyes, causing blurred vision.
  
• Nystagmus, jerky eye movements, occurs in some people.
  Complaints of vision, strabismus or double vision due to pressure on the 6th cranial nerve.
  
• Balance problems and drunkenness. The tumor is located close to the part where balance is regulated: the cerebellum. The
  
• Drunken walking that can then occur is called ataxia in medical terms.
  

Read more about medulloblastoma via this link

Pineal Gland Tumors

See all explanations on our the special page on consequences per brain area. 

Pineoblastoma/pinealoblastoma Grade IV
A blastoma is a precursor of brain cells. These cells should have developed into brain cells. Therefore, this tumor mainly occurs in children.

Brain cyst

A brain cyst is a fluid-filled cavity in the brain. Because the skull cannot expand as the cyst grows, it can put pressure on the brain tissue, causing increased brain pressure.
Increased brain pressure can lead to complaints of:

• headache
  
• vision problems (strangular papilla)
  
• drowsiness
  
• dizziness
  
• nausea and vomiting
  
• epileptic attacks
  
• Hydrocephalus
  

Due to the pressure on the brain, it can lead to symptoms of arm and/or leg failure and disturbed coordination of movements or speech disorders.

A cyst does not always cause symptoms, but can be discovered accidentally on a scan made for another reason.
This usually concerns arachnoid cysts that can be present before birth and can cause symptoms at any age, but usually cause symptoms before the age of ten. It concerns boys more often than girls.

An arachnoid cyst can also be the result of a brain tumor, meningitis or subarachnoid hemorrhage.

Bridge angle tumor (Vestibular Schwannoma)

A tumor of the sheath (nerve sheath) of the auditory
and / or the vestibular nerve is called a transverse angle tumor (vestibular schwannoma or acoustic neuroma).
It is a benign tumor named after the location in the skull: the bridge angle. The bridge angle is located at the back of the skull, in the base of the skull and is located in the angle between the small brain (the cerebellum) and part of the brainstem, the pons.
The image below shows a one-sided bridge angle tumor.

By RadsWiki - RadsWiki, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3520114

The tumor arises from so-called Schwann cells.

The Schwann cell is a special form of glial cell. It forms a sheath and a supporting cell of the 8th cranial nerve (vestibulocochlear nerve) that carries information about sound and balance (balance) from the inner ear to the brain.
Schwann cells only exist in the peripheral nervous system. In the central nervous system, oligodendrocytes perform the same function.
Most people with a bridge angle tumor experience one-sided hearing loss and a feeling of uncertainty when walking (balance disorder). If the transverse angle tumor occurs on both sides, it is considered the hallmark and main criterion of neurofibromatosis type II (NF2), a genetic disorder.

Symptoms of a transverse angle tumor can vary, depending on whether pressure develops on surrounding nerve pathways or on brain tissue.
The growth rate or size of the tumor does not always have implications on the severity of the complaints:

• poor hearing
  
• problems with balance (uncertainty when moving)
  
• headache
  
• disturbances in facial expressions (facial paralysis (Bell's palsy) or spasms) due to pressure on the seventh cranial nerve (facial nerve)
  
• inability to close one of the eyes properly
  
• drooping corner of mouth
  
• change of taste
  
• a numb feeling in the face (trigeminal hypoesthesia) due to pressure on the fifth cranial nerve (trigeminal nerve)
  
• facial pain (trigeminal neuralgia) due to pressure on the fifth cranial nerve (trigeminal nerve)
  

In rare situations, the tumor can become so large that a
hydrocephalus develops. This is caused by a blockage of the cerebrospinal fluid circulation. Cerebrospinal fluid is normally produced permanently in the brain brain cavities (ventricles) and refreshed frequently. That is a serious situation. 
It may cause the following complaints:

• headache
  
• drowsiness
  
• vomiting
  
• see poorly
  

In the rare situation that the tumor grows so large that it presses against the brainstem, this is also a very serious situation. Complaints may then relate to important life functions such as heart rate, breathing and blood pressure. However, complaints of a different nature occur earlier as the tumor grows.

There are basically three treatment options that must be viewed individually and depend on various personal factors:
1. wait and check with an MRI scan once a year (wait and scan)
2.operate (through the ear, above the ear or behind the ear)
3. stereotactic irradiation / 'radiosurgery'
More information can be found here:

Oligodendroglioma
An oligodendroglioma is a rare brain tumor. It arises in a special type of cells in the brain and spinal cord (oligodendrocytes). These cells produce the conductive layer that surrounds nerve fibers (myelin sheath).
Low-grade tumors (grade 2). There is usually slow growth and the tumor does not spread quickly. Complaints often arise late.
High-grade tumors (grade 3 and 4). These oligodendrogliomas are highly malignant. There is rapid tumor growth and the tumor spreads quickly.
These tumors are also called anaplastic oligodendrogliomas.