Craniopharyngioma is a benign cystic–epithelial neoplasm localized in the area of the Turkish saddle, which develops from the remnants of the epithelium of the Ratke pocket. As the tumor progresses and grows, the patient experiences an increase in intracranial pressure, visual impairment, lag in physical and mental development, hypofunction of the adenohypophysis. The most informative methods of diagnosis of craniopharyngioma are MRI and CT, which helps to determine the structure, boundaries of the tumor and the presence of calcifications. Treatment is surgical, after which the patient is prescribed a course of radiation therapy aimed at stopping the further growth of the neoplasm.
ICD 10
D44.4 Neoplasm of uncertain or unknown nature of the craniopharyngeal duct
General information
Craniopharyngioma is a benign brain tumor that originates from the embryonic period and, as a rule, is located in the hypothalamic-pituitary region. As the neoplasm progresses, cysts filled with a liquid with a high content of cholesterol and proteins can form in its tissues. The disease is more inherent in children, however, it can appear at any age.
The incidence of craniopharyngiomas is approximately 2-3% of all types of brain tumors. Neuroepithelial craniopharyngioma is most common (about 10% of cases), and the peak of the disease occurs at the age of 5 to 15 years. Another type of craniopharyngioma is papillary, which occurs most often in people after 40 years.
Causes
The main causes of the development of craniopharyngioma are heredity and various kinds of mutations. In addition, the development of pathology is influenced by adverse factors, especially during the laying of the main organs – the first trimester of pregnancy. The causal factors of the occurrence of craniopharyngioma include the influence of drugs, toxins, poisons, radiation, etc.; intrauterine infection, severe early toxicosis, chronic diseases of a pregnant woman (tuberculosis, diabetes mellitus, kidney failure).
Craniopharyngioma is divided into 2 types: adamantinomatous and papillary. Approximately 60% of craniopharyngiomas are cystic formations, about 15% are solid formations, the remaining 35% have a mixed structure and structure.
The craniopharyngioma develops from the embryonic remnants of the Rathke pocket, a structure from which the adenohypophysis subsequently forms and develops. The neoplasm in most cases has cysts, and in adults it contains calcifications. Due to its localization and progression, craniopharyngioma causes developmental delays in children, visual function disorders, diseases of the endocrine system in adults.
Craniopharyngioma symptoms
Craniopharyngiomas may not manifest clinically for a long period of time. Most often, the first symptoms of the disease occur closer to 5-10 years. The primary manifestations that force the patient to consult a doctor are constant headaches that are not relieved by analgesics, neurological disorders, disorders of the endocrine system.
As the tumor progresses, it begins to squeeze the hypothalamus, pituitary gland and visual intersection, which makes the clinical symptoms more pronounced and vivid. The most typical clinical manifestations of craniopharyngioma are obesity and short stature.
The progression and growth of the tumor causes deterioration of the patient’s vision at the initial stage, which doctors pay attention to during the initial examination of the patient. At a later stage of the development of the disease, an ophthalmologist’s examination with ophthalmoscopy reveals irreversible changes in the fundus, edema and atrophy of the optic nerve. The severity of the clinical picture largely depends on the size and degree of progression of the neoplasm.
The main sign of craniopharyngioma, which is determined only by X-ray examination of the skull, is a change in the Turkish saddle. The picture clearly shows the thinning of its back and wedge-shaped processes.
Diagnostics
Diagnosis of craniopharyngioma consists in carrying out an instrumental modern diagnosis and consultation with a neurologist. Skull x-ray reveals calcifications in the tumor, erosion of its walls and an increase in the size of the Turkish saddle. It is mandatory for the patient to undergo a series of tests to detect the amount of hormones in the blood (examination of the level of SMT, ACTH, TSH, cortisol and thyroid hormones).
The most informative and accurate methods of diagnosis of craniopharyngioma are MRI and CT of the brain. With the help of these research methods, the specialist manages to obtain a layered image of brain tissues, which makes it possible to most accurately determine the location of the pathological process, the size of the formation, the structure and shape of the tumor, its location relative to the surrounding tissues.
Craniopharyngioma must be differentiated from other tumors of the pituitary region: pituitary adenoma, chiasmal glioma, germinoma, as well as with colloidal cyst of the III ventricle. Accurate differentiation of tumors is possible only after histological examination of their tissues.
Treatment
Treatment of craniopharyngioma is performed surgically and is carried out by neurosurgeons. The path of access to the tumor largely depends on its location and size. If complete removal of the neoplasm is impossible for any reason, then radiation therapy methods are used in the postoperative period.
Resection of craniopharyngioma eliminates the pressure of the tumor on the pituitary gland and neighboring brain tissues, and with the help of radiation therapy, it is possible to restrain the progressive growth of the tumor in 90% of cases. A modern and effective method of treating craniopharyngioma is the drainage of the cyst and the introduction into its cavity of an antibiotic that has a detrimental effect on tumor tissues and cells (bleomycin).
Prognosis and prevention
The prognosis in most cases depends on the timeliness of the diagnosis and the experience of doctors during surgery. Relapse of the disease occurs in the first 3 years after surgery and occurs, as a rule, with non-radical removal of the neoplasm. One of the complications in the postoperative period is the development of diabetes insipidus, so the patient needs long-term hormone replacement therapy under the supervision of an endocrinologist.
In addition, the use of radiation therapy, although it is an effective way to stop the further growth and progression of the tumor, causes mental retardation in children and severe lesions of internal organs, in particular the liver. Prevention of the pathological process consists in the antenatal protection of the fetus.
