Cerebral Cavernoma

Generality

Cerebral cavernoma is a benign tumor located in the brain or spinal cord, consisting of a cluster of blood vessels and resembling a raspberry.

Often from unknown causes, brain cavernomas can be completely asymptomatic (in most cases) or cause different symptoms, such as: epilepsy, cerebral haemorrhage, neurological deficits of various nature or headache.

Depiction of a brain cavernoma; note the tangle of blood vessels that characterizes this benign tumor

The therapy depends on the presence or absence of symptoms and the severity of the latter.

Brief reminder of what a hemangioma is

A hemangioma is a benign tumor of endothelial cells, which cover the inner walls of blood vessels.

Consisting of a dense agglomeration of capillaries and blood vessels of a larger caliber, a hemangioma can have the appearance of a patch (or swelling) of purplish color and localization of the skin ( capillary hemangioma ); in other cases, the hemangiomas resemble nodules, always purple in color but capable of forming also on the internal organs - such as the liver - and in the deep states of the skin ( cavernous hemangioma or cavernoma ).

Hemangiomas can appear anywhere, however there are more affected anatomical sites than others: head, cheeks and neck, as far as capillary hemangiomas are concerned; brain, eyes, heart, spleen, respiratory airways and liver, as far as cavernous angiomas are concerned.

The word hemangioma derives from the union of three Greek terms: haema- ( αίμα ), blood; angeio ( αγγείο ), vase; -oma ( -ωμα ), tumor.

What is a cerebral cavernoma?

Cerebral cavernoma - also known as cerebral angioma or cerebral cavernous angioma - is a cavernous hemangioma located in the brain or, more rarely, in the spinal cord.

Constituted by a tangle of blood vessels and having the appearance of a raspberry, cerebral cavernomas are generally single benign tumors, whose dimensions can vary from a few millimeters to 3-4 centimeters.

What is a benign tumor?

A benign tumor is an abnormal mass of proliferating cells that, unlike what happens in malignant tumors, is neither infiltrating nor metastatizing (ie it does not spread its own cancer cells, more properly called metastases, to other organs or tissues of the body).

ARE THERE SOME AREAS OF THE ENTHUSAL MORE THAN HIT THE OTHER?

In the brain, the cerebral cavernoma can appear anywhere; however, there are areas more affected than others, such as the telencephalon (or brain ) and the brainstem .

NB: the brain is one of the two components of the central nervous system (the other is the spinal cord) and consists of: telencephalon (or brain), diencephalon, cerebellum and brainstem.

IS CEREBRAL CAVERNOMA DANGEROUS?

To date, no cases have been observed in which a malignant brain tumor has arisen from a cerebral cavernoma. This, however, does not exclude the possibility that brain angiomas can cause problems that can negatively affect the quality of life of the affected person.

Epidemiology

According to some Anglo-Saxon scientific studies, the cerebral cavernoma has the same frequency in both sexes and equally affects all the races of the world.

Causes

The precise causes of cerebral cavernomas are, at the moment, unclear.

In most cases, the origin of brain angiomas is completely unknown and undecipherable.

More rarely, it can be associated with a certain family predisposition or a previous radiotherapy treatment .

CEREBRAL CAVERNOMES AND FAMILIARITY?

From several scientific studies, it has emerged that a small number of patients with cerebral cavernoma have one or more relatives with the same benign tumor.

This observation led doctors and researchers to formulate a genetic hypothesis, according to which the presence of brain angiomas, in some individuals, would be due to genetic factors transmitted from parents to children.

Although they deserve further study, some research reports that an individual's child with a cerebral cavernoma has a 50% chance of developing the same benign tumor.

CEREBRAL CAVERNOMES AND RADIOTHERAPY

Studying the cerebral cavernoma and its incidence, some scientists have found that several sick individuals had been subjected to radiotherapy in the past. This has led to thinking that there may be a certain relationship between the high-energy rays used in radiotherapy treatment and the appearance of a cerebral angioma.

However, this hypothesis, carried out in recent years by different study groups, presents some question marks; above all, the fact that, for a proportion of the individuals considered, there is no possibility of comparing brain conditions before radiotherapy treatment (in other words, there is no way of knowing whether, before radiotherapy, cerebral angioma was already present or not).

Symptoms and Complications

Most individuals with cerebral cavernoma are fine and do not report any disturbances related to this condition; in all these cases we speak of asymptomatic cerebral cavernoma .

Epidemiology of asymptomatic cerebral cavernoma

From an Anglo-Saxon statistical research, it emerged that, in the United Kingdom, one individual out of every 600 people has an asymptomatic cerebral angioma (ie not accompanied by obvious manifestations). However, since benign tumors are often diagnosed by chance in such situations, the actual number of affected individuals is probably higher.

When present, the symptoms and signs of a cerebral angioma ( symptomatic cerebral cavernoma ) may consist of:

• Attacks of epilepsy . Often, it is just after an attack of epilepsy (usually the first) that the patient undergoes tests and discovers that he has a cerebral angioma.
• Progressive or transient neurological deficits, whose appearance occurs due to the pressure exerted by the benign tumor mass on adjacent nerve structures. Some of the possible neurological deficits are:
• Double vision and / or blurred vision;
• Problems of balance and instability;
• Problems of sensory perception and sense of numbness in various parts of the body;
• Sense of weakness;
• Paralysis of a more or less extended part of the body;
• Speech difficulties (dysarthria).
• Hemorrhagic stroke . This condition occurs when a blood vessel of the cerebral angioma, located in the brain, breaks or suffers a blood loss, resulting in a cerebral hemorrhage. The symptoms of a cerebral hemorrhage are:
• Headache
• Nausea
• Loss of knowledge
• Neurological deficits of various kinds (generally similar to those indicated above)
• Headache . These are transitory episodes, due to the pressure that a cerebral cavernoma, located in the brain, exerts on the nearby bone structures of the skull.

Epidemiology of symptomatic cerebral cavernoma

According to the same Anglo-Saxon statistical research referred to previously, in the United Kingdom, the annual incidence of symptomatic cerebral cavernoma was equal to one case per 400, 000 people.

Warning: it is not excluded that a brain cavernoma that has always been asymptomatic, can suddenly become symptomatic.

WHAT DO NEUROLOGICAL DEFICIT DEPEND ON?

Neurological deficits, which may arise due to a cerebral angioma, depend on the region of the brain or spinal cord involved. In fact, it is recalled that, to different areas of the brain and spinal cord, different body districts and different motor and non-motor faculties (for example, the right hemisphere of the brain controls the word and language, therefore its own interest causes difficulty in articulating a meaningful and clear language).

WHY A CEREBRAL CAVERNOMA COULD BREAK AND LOSE BLOOD?

A cerebral cavernoma is a tangle of blood vessels with thin (sometimes very thin) and delicate walls.

Radiological image of a cerebral cavernoma after a recent blood loss. From the site: www.neurochirurgia-ire.it

It is precisely these vessel characteristics (subtlety and delicacy) that make the brain angiomas predisposed to blood loss.

CEREBRAL HEMORRHAGE AND GRAVITY? OF SYMPTOMS

If the angioma blood loss is minimal, it is likely that the resulting manifestations are minor. If, however, the cerebral hemorrhage is conspicuous, the effects will most likely be more evident and of greater gravity.

Therefore, when there is blood loss from the cerebral cavernoma, each patient represents a case in itself.

What is the probability of developing bleeding?

After several researches, doctors and scientists concluded that:

• For those with cerebral cavernoma who have never been victims of cerebral haemorrhages (obviously linked to angioma), the probability of a blood loss from the benign brain tumor is less than 1%.
• For those with cerebral cavernoma who have suffered a brain haemorrhage in the past, the likelihood of another bleeding from the angioma is between 4 and 25%.
• With the passage of time and if there is no blood loss of any kind, the risk of a second cerebral hemorrhage is progressively reduced.

GREATNESS OF CEREBRAL CAVERNOMA AND SYMPTOMS

The larger the size of the cerebral cavernoma, the more probable it is that it causes obvious and severe disturbances. However, there are people with small brain angiomas who still have significant problems.

WHEN TO REFER TO THE DOCTOR?

The appearance of sudden and recurrent headaches or, worse still, of various types of neurological deficits (visual, motor, etc.) must induce the subject, affected by the ailments, to contact his doctor for a deepening of the situation.

COMPLICATIONS

If a cerebral cavernoma that causes serious symptoms (characterized, for example, by severe hemorrhage) is not treated properly, it can lead to the onset of persistent and increasingly debilitating neurological problems.

Therefore, it could significantly influence the quality of life of those who are carriers.

Diagnosis

The most useful diagnostic tool in highlighting a cerebral cavernoma is nuclear magnetic resonance ( NMR ), a very specific and completely harmless test for the patient.

As an alternative to MRI, doctors can prescribe a CT ( computed tomography ), which, however, exposes the individual under investigation to a minimal dose of harmful radiation.

The objective examination - which is generally carried out before the MRI (or TAC) and consists in the analysis of the symptomatology - is not sufficient to establish the exact nature of the pathological manifestations.

Rights and limits of MRI and TAC

MRI and CT are able to provide clear images of the brain or spinal cord, including any anomalies (such as benign or malignant tumor masses).

However, they present a rather important limit: from their reproduction it is impossible to predict whether a cerebral cavernoma is predisposed to break and to give cerebral hemorrhage.

Treatment

Radiological image of a cerebral cavernoma.

From neurocirugiacontemporanea.com

Therapy depends on the presence or absence of symptoms and their severity.

In the case of asymptomatic cerebral cavernoma, the medical advice is to monitor, with periodic MRIs, the status of the benign tumor.

In the case of cerebral cavernoma marked by sporadic attacks of epilepsy and headaches unrelated to bleeding episodes, doctors prescribe appropriate pharmacological treatments to alleviate these symptoms.

In the case of cerebral cavernoma characterized by cerebral hemorrhage and / or severe neurological deficits, doctors resort to an invasive solution such as surgery, aimed at removing the benign tumor .

There are two adoptable surgical procedures:

• The neurosurgery operation. Made under general anesthesia, it involves the incision of the skull and the removal of the benign tumor (when it is found in the brain).
• The operation of stereotactic radiosurgery . The elimination of cerebral angioma occurs by exposing the benign tumor mass to a beam of high-energy ionizing radiation. General anesthesia is not provided.

NEUROSURGERY OR RADIO SURGERY?

The neurosurgery operation is the most practiced procedure, because it has generally better results.

Figure: the famous AS Roma player, Leandro Castan, underwent an operation to remove a cerebral cavernoma in November 2014.

However, if the cerebral cavernoma is located in an uncomfortable location, it is preferable (also for safety reasons) to resort to radiosurgery.

Prognosis

The prognosis varies from case to case, depending on the severity of the symptoms. Those who need surgery may experience various complications, some of them even fatal.

The risk of surgical complications basically depends on the site of the cerebral cavernoma.