Craniopharyngioma is a benign cystic epithelial tumor of the “Turkish saddle” area. The alleged causative agent of the formation of this tumor is called the remnants of the epithelium of the Ratke pocket. Neoplasm refers to slow-growing. It is localized in the capillary and suprasellar regions, and occurs in the pituitary funnel, gradually spreading into the hypothalamus. The tumor can grow in any of the areas of the craniopharyngeal duct, which stretches from the pharynx to the “Turkish saddle” and the third ventricle.
The content of the article:
Causes of craniopharyngioma
Symptoms of craniopharyngioma
Diagnosis of craniopharyngioma
Treatment of craniopharyngioma
Prognosis and prevention
Craniopharyngioma
During the progression of the neoplasm, cysts filled with yellow-brown liquid with a high concentration of proteins and cholesterol most often appear. Sometimes this neoplasm can reach gigantic sizes and affect nearby anatomical structures — the anterior and middle cranial pits. In some cases, craniopharyngioma may be localized in the area of the optic nerve, pineal gland, bridge of the cerebellar angle.
This type of tumor is more common in children, but can manifest itself at any age. It is generally believed that there are two peaks of the disease — at the age of five to fifteen and from fifty to seventy years. The frequency of the disease does not depend on the gender of the patient.
Craniopharyngioma occurs in about three percent of patients diagnosed with a brain tumor. The most commonly diagnosed type of craniopharyngioma of the brain is neuroepithelial (about ten percent of all cases of brain tumors). Such a tumor is more common in children (6-16 years old). Another type of craniopharyngioma is papillary. It is most often observed in elderly patients (50-70 years old) and is localized in the area of the third ventricle.
Causes of craniopharyngioma
The main reason for the appearance and development of this type of tumors is heredity and various mutations of chromosomes. The development of pathology can also be provoked by additional negative factors, especially those that had an impact in the first trimester of pregnancy, when the formation of the main organs of fetal vital activity took place. This may be the effect of medications prohibited for use during pregnancy, the effects of poisons and toxins, as well as radiation radiation on the body of a pregnant woman.
Intrauterine infection, chronic diseases of the expectant mother (tuberculosis, kidney disease, diabetes mellitus) can also act as a provocateur of the formation and development of this pathology or severe early toxicosis.
At the cellular level, the cause of the formation of craniopharyngioma of the brain is called excessive overgrowth of the epithelium of the Ratke pocket, which is formed in the first trimester of embryogenesis and subsequently gives the basis to the pituitary stalk. If the processes of embryogenesis are normal, then after the formation of the pituitary gland, the cells of the Ratke pocket no longer appear. If these cells continue their division, then this provokes the formation of a craniopharyngioma.
Symptoms of craniopharyngioma
The clinical picture of craniopharyngioma may not manifest itself for a long time or manifest itself as weak signs characterizing the growth of the neoplasm. The symptoms of this type of tumor are often purely individual in nature and depend on many factors, primarily on the characteristics of the patient’s body and his chronic diseases. Bright symptoms are most often manifested in patients aged ten to twenty years. More than half of these tumors have the form of a cystic neoplasm, a little more than ten percent are solid tumors, there are also craniopharyngiomas of mixed type.
If a person develops craniopharyngioma of the brain, then, first of all, there are disorders in the endocrine system (disorders of the adrenal glands, diabetes insipidus, menstrual cycle disorders in women, potency in men). Pain syndromes are also noted, vision decreases, signs of hydrocephalus may occur (due to a violation of the function of transporting cerebrospinal fluid in the area of the third ventricle), as well as neuropsychological disorders. In adolescence, with the development of a tumor of this type, puberty is delayed or vice versa — its development is too early and very rapid.
Headache, which is one of the first signs of brain tumors, in this case is characterized by an increasing character. Visual disturbances are associated with the growth of the tumor body, which squeezes the optic nerve. Over time, the infringement of the optic nerve can lead to its complete atrophy.
Neuropsychological disorders can manifest as dementia, bulimia, or severe and moderate forms of obesity. There may also be inhibition, deterioration of motor skills, emotional instability, apathy, violation of short-term memory.
In children, the symptoms of craniopharyngioma are most often expressed in growth retardation.
Diagnosis of craniopharyngioma
Diagnosis of craniopharyngioma begins with a consultation with a neurologist and oncologist. Next, an X-ray of the brain is prescribed, which reveals the structure characteristic of the tumor and the erosion of its walls, as well as the corresponding changes in the structure of the Turkish saddle. Calcified tumors are clearly determined by such studies.
Laboratory tests are also carried out to detect the concentration of hormones in the blood.
The tumor is diagnosed using MRI and CT scans of the pituitary gland of the brain, which allow you to see the layers of brain structures and determine not only the localization of craniopharyngioma, but also its size, which is extremely important.
Sometimes dopplerography of cerebral vessels is prescribed, as well as angiography of the brain and spinal cord.
To make an adequate diagnosis, craniopharyngioma should be differentiated in a number of other tumors with similar symptoms, for example, pituitary adenoma, glioma, colloidal cyst of the third ventricle.
Treatment of craniopharyngioma
Craniopharyngioma belongs to a number of benign tumors, so it has several treatment options:
Radiosurgery using cyber knife and gamma knife technology is used in clinics in Israel and Germany to completely remove the tumor. Radiation therapy is used to prevent the progression of the tumor. After radiation therapy, the patient should be monitored for about five more years and twice a year to perform MRI and CT to control the volume of the tumor.
The most traditional method of treating craniopharyngioma is surgery. Especially relevant for large volumes of the tumor, when the use of radiosurgery is impossible. In the treatment of craniopharyngioma, the operation gives the most encouraging prognoses.
Very often, it is impossible to completely remove the tumor due to the dense location of the neoplasm body to the optic nerves and other vital structures. After the operation, the cyber knife technique is used or the remnants of the tumor are removed using radiation and hormone therapy.
Hormone therapy is used as a method of stabilizing the disruption of pituitary hormones.
Prognosis and prevention
The prognosis of a tumor, as in most other cases, depends on many factors, including timely diagnosis and provision of adequate treatment. In some cases, a relapse is not excluded (especially with incomplete removal of the tumor body) and the progression of the disease, which can provoke a number of diseases and disorders in the body — diabetes insipidus, obesity, etc.
The best prevention of craniopharyngioma is a healthy lifestyle of a pregnant woman and compliance with all contraindications during the period of carrying a child.
