The incidence of cancer on our planet is growing from year to year. Such pathologies affect a variety of parts of the human body. Craniopharyngioma of the brain is a disease in which the nervous system inside the skull suffers. The frequency of occurrence is from one to two cases per million people.
General information
Everyone should know what a craniopharyngioma of the brain is. Although the disease is rare, no one is immune from it. The term refers to a pathological condition that is formed even before the birth of a child. A congenital disease is explained by the abnormal structure of the tissues of the embryo in the region of the pituitary pocket. The oncological process belongs to the category of benign. The disease is classified as disembryogenetic. Tumor processes are triggered if the formation of the epithelium in the embryo is disrupted, while failures affect the craniopharyngeal duct.
Lastprovides a connection between the brain structures and the pharyngeal cavity. As the fetus develops, this area gradually overgrows. Some fail, leaving the passage open. The epithelial tissues covering it on the inner surface, with this development of the organism, turn into a substrate for the tumor process.
Anatomy and biology
A study of different cases shows that craniopharyngioma of the brain in adults and children is observed with different features of the topography. When evaluating a case, it is necessary to check its localization relative to the Turkish saddle. In some, the pathology is located inside the plane, in others it is located directly above it. Another possible option is a wall behind the Turkish saddle.
Endosuprasellar - such a variant of the location of the pathological process, which is more often diagnosed with a large neoplasm. The tumor fills the Turkish saddle and even becomes somewhat higher than it. The formation puts pressure on the brain tissue and the nervous system responsible for its nutrition, compresses the CSF pathways. Over time, this leads to specific symptoms. The most dangerous situation is when tissue remnants press on the optic nerve. No less severe case - accompanied by the germination of tumor tissue in the third cerebral ventricle.
Causes and factors
The tumor is formed by a fairly dense tissue. The diameter of the neoplasm is from a couple to five centimeters. At the moment, scientists do not know the causes of the disease. It is assumed thatany pathological factor, due to which the development of the embryo is disturbed, can provoke an oncological disease. If the mother smoked and drank alcohol, took medications or faced increased stress, the child she gave birth to could develop such a pathology. It can be provoked by maternal overload and malnutrition. The risk of contracting a benign neoplasm is higher in those who encountered an infectious agent during fetal development. Additional risks are associated with toxicosis in the early period of gestation.
Clinical manifestations
You can notice the characteristic symptoms of the disease in a newborn, a small child, an adult. Symptoms of craniopharyngioma of the brain are malfunctions of the nervous system that feeds the brain. Most of them have a malfunction of the optic nerve. The ability to see weakens, the fields of vision gradually become narrower. For some, the development of the process is such that it affects the state of the nervous system, which provides the ability to recognize aromas. In this course, a person completely loses his sense of smell or begins to perceive smells incorrectly. From medical practice, there are cases of patients suffering from strabismus due to oncological disease. There is a danger of neuralgia affecting the trigeminal nerve.
About symptoms in more detail
Symptoms of brain craniopharyngioma include a malfunction of the pituitary gland. In people,suffering from cancer, the development of this area is blocked. Organic tissues are not mature enough, which leads to the impossibility of normal functioning of the internal secretory systems. This affects numerous glands. Such a developmental disorder is especially noticeable in children - growth lags behind peers, a vegetative crisis is observed. Many children suffering from this disease face difficulties in natural thermoregulation.
Symptoms: pay attention
A severe variant of the course of the pathology is the spread of the tumor process to the ventricles of the brain. This development of the situation affects the ability of a person to sleep, and the violations are very persistent.
In pathology, there is a cerebral syndrome. Craniopharyngioma can be suspected by increased intracranial pressure and hydrocephalus. Hypertension is indicated by headaches. The patient describes the syndrome as bursting. Many people vomit but don't get better after that.
Hydrocephalus
Craniopharyngioma of the brain in children and adults can cause hydrocephalus. The pathological process leads to damage to the organic tissues that form the brain. The functionality of the cortical layer is disturbed, the higher brain function suffers. About half of the patients with the described diagnosis, who have been struggling with pathology for a long time, are faced with a persistent decrease in intelligence. Patients are characterized by personality changes. Many people have symptomatic epilepsy. Slightly less commonly diagnosed disorderssensitivity. Possible movement problems.
The peculiarity of the course of the disease in question is a wave-like character. The stabilization period is quite long, but can suddenly change to a pronounced progress of the disease.
Clarification of the diagnosis
In adults, in children, craniopharyngioma of the brain is a disease, if suspected, the patient must be carefully examined. Diagnostic measures should be comprehensive. First, the patient is interviewed, then sent to a neurologist for a detailed examination. The conclusion of the endocrinologist is necessary. The patient should visit an ophthalmologist. Doctors will select instrumental methods of examination. One of the most important is neuroendocrine. Pathology is accompanied by insufficiency of the pituitary gland, hypothalamus, strongly affects the functioning of the adrenal glands, thyroid gland. To determine the features, it is necessary to do a screening to identify the nuances of the hormonal profile.
Examination by an ophthalmologist almost always shows an increase in pressure inside the skull. Assessing the condition of the fundus, the doctor identifies congestive processes. Perimetry allows you to determine the narrowing of the fields or the complete loss of individual sections from the susceptibility zone. In many patients, the ability to distinguish and identify colors is impaired, vision becomes weak, and the phenomenon is often asymmetrical.
Check everything
If a craniopharyngioma of the brain is suspected, the patient is prescribed a CT scan of the brain. The most informative optionMRI. The results of the instrumental examination give a general idea of the structure of the bone and soft tissues that form the organs. A tomogram allows you to identify a tumor formation, evaluate the features of its shape and the nuances of localization.
MRI results give an idea of the dimensions of the pathological process. From them you can understand in which direction it develops. Layered images reflect the structure of the tumor, give an idea of the increased pressure inside the skull and its severity. If the MRI shows a formation with dense walls and internal petrifications, there is no doubt about the reliability of the diagnosis.
Features of the case
With craniopharyngioma of the brain, a course is possible that is not accompanied by the generation of calcifications. The contents of Rathke's pocket may be homogeneous. To make the most accurate diagnosis, it is necessary to refer the patient to additional research activities.
If the pathological process is large, the formation can put pressure on the cranial bones. In this case, the pictures will show atrophied areas of the cranium. Similar changes may cover elements of the Turkish saddle.
What to do?
Treatment of craniopharyngioma of the brain is chosen based on the characteristics of the disease, according to the current classification system, classified as primary in terms of malignancy. To improve the patient's prognosis, it is important to start a therapeutic course in a timely manner. Choose a comprehensive program. The patient was recommended surgery and conservative medical treatment.help. Before surgery, you must first be treated briefly with hormonal drugs in order to minimize the likelihood of complications. Treatment for cerebral craniopharyngioma involves shunting if the hydrocephalus is severe and accompanied by obstruction. Such a measure reduces the pressure inside the skull, thereby reducing the damage received by the brain.
Stereotactic techniques are chosen for operation. Cranial trepanation is allowed. Rathke's pocket is cleared of filling. To remove residual tissue and prevent recurrence, the capsule is filled with bleomycin, a substance that activates sclerotic processes.
Using an endoscope minimizes brain damage. Such an event as part of the treatment of cerebral craniopharyngioma is much better tolerated. In recent years, methods of radiological surgery have become widespread, in which radioactive isotopes are injected into the brain.
If classic surgery is chosen, osteoplastic trepanation is recommended.
Operation: what's next?
According to reviews, brain craniopharyngioma is a disease that is difficult to treat. To improve the prognosis after surgery, the patient is prescribed a hormonal therapy program that can reduce the activity of inflammatory foci and reduce the tendency of brain tissues to edema. Diuretics and radiation treatment are put. Although the tumor process is benign, there is a risk of recurrence of the disease and degeneration of cells into atypical ones.
In remote nature after surgery, replacement therapy with synthetic hormones is recommended. Residual effects are varied and depend on the case. For some, vision weakens, the possibility of absolute blindness is not ruled out. In patients, the potency decreases, in women the cycle is disturbed. The consequences of the pathology can be diabetes insipidus and hypothyroidism, adrenal insufficiency. Some retain manifestations of infantilism for the rest of their lives.
Forecast
Determined by the size of the formation, depends on the timeliness of the diagnosis. Survival over ten years is estimated at 40-80%. This applies only to those who underwent surgery on time. At the same time, it is noted that the operation does not exclude recurrence. On average, the disease returns in every fifth patient. Even if it is impossible to completely remove tumor tissues, an improvement in prognosis is achievable by undergoing a radiation course of treatment.
Reviews
As can be concluded from the reviews, craniopharyngioma of the brain is one of the serious diseases. Even those who received appropriate treatment on time often faced dire consequences. Doctors who had experience in diagnosing and treating this disease recognize its problematic nature, complicated by its rarity and low degree of knowledge. There are practically no reviews of people who have coped with craniopharyngioma, since the pathology is extremely rare.
