Craniopharyngioma

Craniopharyngioma (synonyms: Ratchet pocket tumor, pituitary tumor, Erdheim tumor) is a predominantly benign brain tumor. Fertilization (turning into cancer) happens extremely rarely. The tumor arises from embryonic (germinal) cells, and it can happen at any age. Craniopharyngioma is located in the area of ​​the Turkish saddle, at the base of the brain. A tumor can manifest itself by changing intracranial pressure, visual impairment, hormonal disorders, changes behavior (including mental disorders), and may not even make itself felt at the initial stages. To detect this disease, a CT scan or magnetic resonance imaging of the brain is necessary. The treatment consists in the removal of the tumor in an operative way, and also in treatment with the use of radiotherapy. All that is associated with this mysterious tumor, you can learn by reading this article.

What is craniopharyngioma

Craniopharyngioma as an independent disease is known since the middle of the XIX century. Then she was discovered by the pathologist at an autopsy accidentally. In 1904, the tumor was described by Erdheim (Erdeym) as a separate type of brain tumors. In 1922 American neurosurgeon H. Cushing suggested the term "craniopharyngioma", which stuck firmly to this tumor, as it is called to this day. This name was given not by chance. The fact is that the tumor is formed from the germ cells that form the cranio-pharyngeal (craniopharyngeal) duct in the embryo at the earliest stages of pregnancy. Normally, after the process of formation of the brain tissues, the unnecessary cells of the cranial-pharyngeal duct undergo a reverse development, that is, as it were, dissolve. If this does not happen, then later, craniopharyngioma develops from these cells.

According to statistical data, craniopharyngioma is about 1-4% of all primary neoplasms of the brain. In children, this indicator is slightly higher and ranges from 5 to 10%. The peak incidence is recorded at the age of 5-16 years and after 50 years. Sexual differences in the structure of morbidity are not revealed, that is, both men and women suffer equally.

There is a tumor at the base of the brain in the pituitary gland. It can have different sizes, but most often 2-5 cm in diameter. The tumor usually has its capsule and does not germinate into surrounding tissues, but simply squeezes them. Due to compression, the main symptoms of the disease appear. Capsule does not allow the contents of the tumor to contact the tissue of the brain, its membranes and nerves. In the event of rupture of the capsule and the expiration of the contents, an inflammatory process develops in the brain substance. Craniopharyngioma - almost always a benign tumor, but due to its anatomical location can give terrible complications.

By its structure craniopharyngioma can be:

• compact (soft) with small cystic cavities and small impregnations of calcium;
• cystic (in the form of a large single or multi chamber cavity filled with liquid);
• rocky (almost completely impregnated with calcium salts);
• mixed structure (combines the features of several varieties).

A distinctive feature of the fluid contained within the cystic craniopharyngioma is a significant content of protein, fatty acids and cholesterol crystals.

There is a classification of craniopharyngiomas, based on their anatomical location. This separation is necessary for planning access in the surgical treatment of this tumor.

The causes of craniopharyngioma

Taking certain medications during pregnancy can contribute to the formation of craniopharyngioma in the fetus.

What causes the cells of the cranial duct to remain viable, and then multiply? Scientists suggest that the wine is served by a variety of harmful factors that affect the fetus during the first trimester of pregnancy:

• use of medicines;
• exposure to toxins or poisons;
• irradiation;
• intrauterine infections;
• the presence of untreated chronic diseases in the mother;
• severe toxemia in early pregnancy.

All these factors can lead to an incorrect laying of fetal organs and systems, including the cranio-pharyngeal duct. However, it should be understood that it is not at all necessary, for example, that after the mother takes any medication her baby will develop craniopharyngioma in the future. Just the risk in general will be slightly higher than that of others.

Symptoms

Craniopharyngioma - a pretty insidious tumor, because at first nothing shows itself. Symptoms appear when the tumor reaches a decent size and begins to squeeze the surrounding tissue. The signs of this tumor are nonspecific, that is, they do not allow you to suspect this particular type of brain tumors. What are these symptoms? The main signs of craniopharyngioma can be:

• headache. This symptom is a reflection of intracranial hypertension. A tumor is always a plus tissue, and the cranial cavity has constant dimensions, so the emergence of additional education inside cranium leads to an increase in intracranial pressure, which in turn makes itself felt headache. The pain can be diffuse, and can be local in the forehead or in the temporal areas, has a noisy character. At first the pain is unstable, but with the growth of the tumor it becomes more and more intense, it acquires a bursting character;
• visual impairment. This symptom is connected with the compression of the visual paths passing near the Turkish saddle. Depending on the direction in which the tumor grows, some or other visual disturbances appear. This can be a partial fallout of the visual fields (when the person does not see the right or left parts of the picture - the so-called hemianopsia), narrowing of the visual fields (when the picture is visible as in a telescope), visual impairment in one eye (blindness). With the long-term existence of craniopharyngioma, atrophy of the optic nerve may develop. In this case, even after removal of the tumor, restoration of vision is impossible;
• symptoms of intracranial hypertension (increased intracranial pressure). They arise as a result of compression of the growing tumor of the third ventricle of the brain, where the cerebrospinal fluid circulates. Because of the violation of the outflow of fluid from the ventricle, it begins to burst the brain tissue from the inside, thereby causing an increase in headache, a change consciousness before stun or sopor, a violation in the mental sphere (inhibition or vice versa, the inadequacy of behavior). In this case, vision can quickly deteriorate (its sharpness decreases), as well as there is an edema of the optic nerve disc (as seen when viewed by the ophthalmologist of the fundus). The intensification of the headache is accompanied by persistent vomiting, which occurs without precursors;
• changes in the mental sphere. Patients can become lethargic, apathetic, indifferent to what is happening around. They seem to be emotionally dull. Memory may deteriorate. Children can lag behind in their neuropsychological development from their peers. Possible sleep disorders;
• hormonal disorders. Craniofaringioma itself has nothing to do with the hormonal background of the body, it does not produce any hormones. But the location of the tumor near the pituitary causes a violation of its function, and the pituitary gland is after all the main endocrine organ of man. The compression of the pituitary gland by a growing tumor can lead to impairment of the sexual glands (menstrual cycle, decreased potency and libido, premature puberty in children or delayed sexual development and growth), the thyroid gland (often a decrease in its function - hypothyroidism, less often - increased function - hyperthyroidism), adrenal glands (tendency to low arterial pressure, changes in hairline, fatigue, intolerance of usual physical activity). In addition, there may be an outflow of breast milk from the mammary glands, obesity or a sharp decrease in body weight. Often the first symptom of craniopharyngioma is diabetes insipidus, when urine is excreted per day to 5-10 liters. Its occurrence is associated with a disruption of the synthesis of the antidiuretic hormone by the pituitary gland. As you can see, hormonal disorders are very numerous and diverse, and at first glance it may seem that they have nothing to do with the brain. But in fact, the growing brain tumor is to blame for everything;
• symptoms of defeat of cranial nerves. Depending on the direction of craniopharyngioma growth in the cranial cavity, it can squeeze I, III, IV, V, VI pairs of cranial nerves. This is manifested by a violation of the perception of odors, a violation of movement of the eyeballs in one or more directions, the dangling of the eyelid, paroxysmal pain in the face;
• epileptic seizures. They rarely become the first symptom of craniopharyngioma, but such a situation is also possible.

From all of the above, it becomes clear that craniopharyngioma does not have any specific clinical manifestations. After all, no headache, no signs of increased intracranial pressure, nor hormonal disorders can not be a reliable proof of its presence. Therefore, the diagnosis of this disease is based not only on complaints and examination data of the patient, but also on additional methods of research, as you will learn below.

Diagnostics

Suspicion of a tumor in the brain occurs when there are certain symptoms (persistent headaches, characteristic changes in vision, the phenomenon of intracranial hypertension, and so on) and data of neurological examination, examination by an ophthalmologist. To confirm or exclude a brain tumor (any, including craniopharyngioma), it is necessary perform computed tomography (CT) or magnetic resonance imaging (MRI) of the brain. Before the advent of these methods of research, radiography of the skull was used, but she could detect only tumors with calcium content. With the advent of CT and MRI diagnostics was significantly simplified. The location of the tumor, some characteristics of its density (in particular, the presence of calcium) suggest that it is craniopharyngioma. To determine its effect on the human hormonal background, it is necessary to determine the concentration of the hormones of the pituitary gland, adrenals, and thyroid gland. Most accurately state that this patient has craniopharyngioma, you can only get the results of a histological examination tumor tissues, since there are also other brain tumors that can give the same symptomatology.

It should be noted that in some cases craniopharyngioma is found by chance during examination for another disease. For example, a person was injured head. To clarify the diagnosis, he is assigned an MRI of the brain, which results in asymptomatic craniopharyngioma.

Treatment

The historical path of medical manipulations with craniopharyngiomas is rather tortuous. The first surgical intervention was carried out as far back as 1909. Halstead. If we take into account the location of the tumor and the level of operational technologies of those times, of course, one can speak of the ineffectiveness of surgical methods of getting rid of the disease. The operations that took place in the first half of the last century were characterized by high rates of mortality and postoperative complications (often becoming causes of death). Therefore, in the second half of the twentieth century, preference was given to radiotherapy. However, radiotherapy only reduced the size of the tumor and for some time stopped its growth, not allowing to get rid of craniopharyngioma forever. The improvement of neurosurgical techniques allowed surgical methods to re-occupy leading positions. To date, surgical treatment is the main one in the detection of craniopharyngiomas. Access to the tumor is carried out in various ways, depending on its location and size. In the postoperative period often resort to radiation methods of treatment, because it is not always possible to completely remove the tumor. Radiation therapy also helps prevent the recurrence of the disease. It is believed that it is impossible to completely remove craniopharyngioma, whose dimensions exceed 5 cm in diameter. Therefore, in most cases, it is necessary to combine surgical treatment with the subsequent radiation treatment.

Another way to get rid of the tumor is to drain the cyst with the introduction into its cavity of the antitumor drug Bleocin or Alfa-interferon.

Among the radiosurgical methods of treatment is the Gamma Knife and Cyber-knife. These are non-invasive methods of irradiating a tumor with thin beams of radiation at different angles. At the same time, healthy brain tissues are practically not exposed to radiation, which minimizes the consequences of radiation treatment. This is possible thanks to stereotaxic techniques, when an accurate calculation of the location of the tumor tissue is made, the radiation beam is fed into this zone, bypassing healthy tissues. Gamma-knife and Cyber-knife can be used as a supplement to surgical treatment of large size craniopharyngiomas, when complete removal is impossible.

In connection with the close arrangement of the important structures of the brain to the tumor, sometimes after total removal of the tumor and radiotherapy there are endocrine disorders (in particular, diabetes insipidus), which require hormonal correction throughout the rest of life. Unfortunately, in some cases, this is the price for getting rid of a tumor. Such patients should receive replacement therapy with appropriate hormones. Sometimes after radiation therapy, hormonal disorders do not occur immediately, so these patients should be on the register and periodically take tests for the content of hormones.

Forecast

Given the location of the tumor, its radical removal at relatively large sizes (more than 5 cm) is impossible. Carrying out after the surgical treatment of radiation therapy can minimize the risk of recurrence, but, unfortunately, not to zero. Most often, relapses occur in the first 3 years. The statistical data on the repeated growth of the tumor are quite contradictory and range from 5 to 35%. The deaths after operations are 5 to 10% (depending on the age of the patients, the size of the tumor, the concomitant diseases).

Although craniopharyngioma is a benign tumor, it is nevertheless located near important structures based on The brain creates the prerequisites for a number of complications arising from the treatment. These complications themselves can subsequently lead to a reduction in life (eg, dementia or diabetes insipidus). It is quite difficult to make a reliable estimate of the survival rate in 5-10-20 years after the removal of craniopharyngioma, because there are only mortality rates in such patients in general (that is, for any reason), and not only for this disease.

Thus, summing up, we can say that craniopharyngioma is one of the brain tumors, the prerequisites to the emergence of which are laid down during pregnancy, and it can manifest itself at any age. Symptoms of this disease are nonspecific and quite diverse, and to clarify the diagnosis it is necessary to conduct CT or MRI of the brain. The choice of method of treatment depends on many factors and is of an individual nature (mainly surgical methods, radiotherapy or a combination thereof). Craniopharyngioma can be defeated!