The frequency of oncological diseases 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 constitutes a craniopharyngioma of the brain. Although the disease is rare, no one is safe from it. The term refers to a pathological condition that is formed even before the baby is born. Congenital disease is explained by the abnormal structure of embryo tissues in the pituitary pocket. The oncological process belongs to the category of benign. The disease is classified as dysembryogenetic. Tumor processes are triggered if the formation of the epithelium in the embryo is disturbed, while the malfunctioning affects the craniopharyngeal duct.
The latter provides a connection between brain structures and the pharyngeal cavity. When the fetus develops, this area gradually overgrows. Some fail, which makes the move open. The epithelial tissues covering it in the inner surface, with this development of the body, 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 topography. When assessing a case, its location relative to the Turkish saddle must be checked. In some, the pathology is located inside the plane, in others it is located directly above it. Another possible option is the wall behind the Turkish saddle.
Endosuprasellar is a variant of the location of the pathological process, which is more often diagnosed with a large neoplasm. A tumor fills the Turkish saddle and even gets slightly higher than it. The formation presses on the brain tissues and the nervous system, which is responsible for its nutrition, compresses the pathways of cerebrospinal fluid. Over time, this leads to specific symptoms. The most dangerous situation is when the remnants of tissue press on the optic nerve. No less severe case is accompanied by the germination of tumor tissue in the third cerebral ventricle.
Reasons and factors
The tumor is formed by a fairly dense tissue. The diameter of the neoplasm is from a couple to five centimeters. Currently, scientists do not know the causes of the disease. It is assumed that any pathological factor, due to which the development of the embryo is impaired, can provoke an oncological disease. If the mother smoked and drank alcohol, took medication, or experienced increased stress, such a pathology may form in her child. Overload of the mother's body and malnutrition can provoke it. There is a higher risk of developing a benign neoplasm in those who in the period of intrauterine development encounter an infectious agent. 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, small child, adult. Symptoms of craniopharyngioma of the brain are malfunctioning of the nervous system that feeds the brain. Most have a violation of the optic nerve. The ability to see is weakening, the field of view is gradually becoming narrower. For some, the development of the process is such that it affects the state of the nervous system, providing the ability to recognize aromas. In this course, a person completely loses his sense of smell or begins to perceive odors incorrectly. From medical practice, cases of patients suffering from strabismus due to cancer are known. There is a danger of neuralgia affecting the trigeminal nerve.
About symptoms more
Symptoms of craniopharyngioma of the brain include a violation in the pituitary gland. In people with cancer, the development of this site is blocked. Organic tissues are not mature enough, which leads to the inability to function properly of internal secretory systems. This affects many glands. Such a developmental disorder is especially noticeable in children - growth lags behind peers, there is a vegetative crisis. Many children suffering from this disease face the difficulties of 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. Such a development of the situation affects the person’s ability to sleep, and the disorders are very persistent.
With pathology, a cerebral syndrome is observed. Craniopharyngioma can be suspected by increased pressure inside the skull and hydrocephalus. Hypertension indicates headaches. The patient describes the syndrome as bursting. Many vomits, but after that it does not get better.
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 impaired, the higher brain function suffers. About half of patients with the described diagnosis, who have been struggling with pathology for a long time, experience a persistent decrease in intelligence. Patients are characterized by personality changes. Many have symptomatic epilepsy. Sensitivity disorders are less commonly diagnosed. Possible problems with movements.
The peculiarity of the course of the disease in question is a wave-like character. The stabilization period is quite long, but suddenly it can be replaced by a pronounced progress of the disease.
Diagnosis
In adults, in children, craniopharyngioma of the brain is a disease, with suspicion of which it is necessary to carefully examine the patient. Diagnostic measures should be comprehensive. First, the patient is interviewed, then referred to a neurologist for a detailed examination. Endocrinologist’s opinion is required. The patient should visit an ophthalmologist. Doctors will select instrumental methods of examination. One of the most important is neuroendocrine. Pathology is accompanied by insufficient functioning of the pituitary gland, hypothalamus, greatly 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.
An examination by an ophthalmologist almost always shows an increase in pressure inside the cranium. Assessing the condition of the fundus, the doctor identifies stagnant 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 asymmetric.
All check out!
If a craniopharyngioma of the brain is suspected, a CT scan of the brain is prescribed to the patient. The most informative option is MRI. The results of instrumental examination give a general idea of the structure of bone and soft tissues that form organs. The 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 is developing. 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 that has dense walls and internal petrificates, there is no doubt about the reliability of the diagnosis.
Case features
With craniopharyngioma of the brain, a course is possible that is not accompanied by the generation of calcifications. The contents of a Ratke 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 images show the atrophied areas of the skull. Similar changes may cover elements of the Turkish saddle.
What to do?
The treatment of craniopharyngiomas of the brain is selected based on the characteristics of the disease, according to the current classification system, which is ranked first in terms of malignancy. To improve the prognosis of the patient, it is important to start a therapeutic course in a timely manner. Select a comprehensive program. The patient is recommended for surgery and conservative medical care. Before surgery, you must first be treated briefly with hormonal drugs in order to minimize the likelihood of complications. Treatment of craniopharyngioma of the brain involves shunting if hydrocephalus is severe and accompanied by obstruction. Such a measure reduces the pressure inside the skull, thereby reducing the damage to the brain.
For the operation, stereotactic techniques are selected. Cranial trepanation is allowed. Ratke's pocket is cleaned from filling. To remove residual tissue and prevent recurrence, the capsule is filled with bleomycin, a substance that activates sclerotic processes.
Using an endoscope can minimize brain damage. Such an event in the treatment of craniopharyngioma of the brain is much better tolerated. In recent years, methods of radiological surgery have become widespread, in which radioactive isotopes are introduced into the brain.
If the classic version of surgical intervention is chosen, osteoplastic trepanation is recommended.
Operation: what after?
According to reviews, craniopharyngioma of the brain is a disease that is difficult to treat. To improve the prognosis after surgery, the patient is prescribed a hormonal therapy program that allows you to weaken the activity of inflammatory foci and reduce the tendency of brain tissue to edema. Diuretics and radiation treatment are laid. Although the tumor process is benign, there is a risk of relapse and degeneration of cells into atypical ones.
In the distant nature after surgery, replacement therapy with synthetic hormones is recommended. Residual effects are diverse and case-dependent. In some, vision weakens, the possibility of absolute blindness is not excluded. In patients, the potency decreases, in women, the cycle is broken. The consequences of the pathology can be diabetes insipidus and hypothyroidism, adrenal insufficiency. For some, manifestations of infantilism persist throughout their lives.
Forecast
It is determined by the dimensions of the formation, depends on the timeliness of the diagnosis. Survival of more than a decade is estimated at 40-80%. This applies only to those who underwent surgery on time. At the same time, it is noted that surgery does not exclude relapse. On average, every fifth patient returns the disease. Even if it is not possible to completely remove the tumor tissue, an improvement in prognosis is achievable by radiation treatment.
Reviews
As can be concluded from the reviews, craniopharyngioma of the brain is one of serious diseases. Even those who received the right treatment on time often faced dire consequences. Doctors who have 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 mastered craniopharyngioma, since pathology is extremely rare.