Craniopharyngioma of the brain in children: symptoms, treatment, removal and prognosis after surgery

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Any cerebrospinal tumor due to its specific localization is regarded by specialists as a very dangerous pathological condition, even though its character is benign. To similar formations it is possible to carry and craniopharygia.

The concept of

Craniopharyngioma is a benign cerebral tumor process developing from embryonic cell structures and localized in the pituitary and hypothalamus regions.

In the international classification of pathologies craniopharyngioma is assigned code D44.4.

With the development of the tumor process in the tissues of craniopharyngoma, cystic fractions are formed, the contents of which are a mixture of proteins and cholesterol.

The most common varieties of pathology are papillary and neuroepithelial craniopharyngioma. Papillary is more typical for middle-aged people after 40, and neuroepithelial is more common in 5-15-year-olds.

According to statistics, more than half of craniopharyngiomas( ≈60%) have a cystic structure, 15% have solid formations, and about a third of tumors are characterized by a mixed structure.

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Causes of development of

Similar tumors are characterized by slow growth. They are congenital and usually appear in adolescence.

This developmental defect is formed by their embryonic rudiments pocket Ratke.

Specialists believe that the main reasons for the development of craniopharyngiomas are:

  • Different kinds of mutations;
  • Hereditary factor;
  • The effect of adverse factors, especially in the first 14 weeks of pregnancy;
  • Effects of poisons and toxic substances, medicines and radiation;
  • Intrauterine infections;
  • Early toxicosis of severe form;
  • Pregnancy has chronic pathologies such as diabetes, kidney failure, tuberculosis, etc.

The tumor is formed from the embryonic cells of the Ratke pocket, later the adenohypophysis is formed from this structure. In adults, along with cystic fractions, craniopharyngioma may contain calcifications.

Symptoms of craniopharyngioma of the brain

In the initial stages, signs of lesions may be completely absent, but with the progression of the pathological process symptomatics begins to manifest itself:

  1. Various endocrine disorders such as diabetes insipidus, hypothyroidism or adrenal disorders, men may noticeably decrease libido and develop erectile dysfunction, andin women, menstruation may disappear;
  2. Intensive headaches usually have an increasing character and are accompanied by pronounced nausea;
  3. Visual disorders occur due to compression of the optic nerve;
  4. Violations of a neuropsychological nature such as obesity, bulimia, inhibition of psychomotor, apathy or dementia, lack of emotionality, incontinence and memory problems;
  5. Hydrocephalus or cerebral edema, accompanied by headaches, visual disturbances or edema of the optic nerve.

Diagnosis

Diagnosis begins with a consultation of a neurologist who assigns the necessary instrumental studies:

  • Radiographic examination of the skull helps to detect erosive processes on the walls of the tumor, calcifications and growth of the Turkish saddle;
  • Laboratory tests of the level of hormones - cortisol, thyrotropic, adrenocorticotropic and somatotropic hormone, as well as hormones produced by thyroid gland;
  • Computer or magnetic resonance imaging. Such studies visualize a layered image of the brain structures and shows the exact localization of the tumor, its parameters and shape, structure, etc.;
  • Biopsy with further histological examination - similar diagnosis allows differentiating craniopharyngioma with other tumor lesions of the brain like germinoma, pituitary adenoma or ventricular cyst, etc.

Treatment of tumor

The approach to the treatment of craniopharyngioma is purely surgical.

If it is impossible to remove the formation completely after surgery, stereotactic or remote radiation therapy is prescribed.

Through the performed tumor resection, it is possible to get rid of the pressure on the nearest tissues, and irradiation allows to restrain tumor growth in the majority of clinical cases.

Very often, specialists combine radiotherapy and surgical methods of treatment, and, if necessary, supplement them with hormone replacement therapy.

When using the radiation method, the entire tumor is exposed to irradiation. For processing, megavoltage radiation, generated by a linear accelerator or a specialized gamma apparatus, is used. Irradiation is usually conducted for 5 consecutive days.

One of the modern methods of treatment today is the drainage of cystic forms by craniopharyngiomas by draining the formation with the introduction of an antibiotic drug into its cavity. Most often for the introduction of Bleocin, which destroys the tumor tissue.

In small formations, the Gamma-knife system is frequently used, which excludes the development of neuroendocrine postoperative complications.

The degree of postoperative functional disorders is determined by the amount of surgical intervention performed. If the tumor was only partially removed, and radiation therapy was not performed, then the formation begins to grow actively and soon reaches its original dimensions.

After radical removal of craniopharyngioma, cases of local recurrence are virtually excluded, although the risk of complications persists.

Usually surgical and radiation treatment is supplemented by hormone replacement therapy with the use of drugs of gonadotropic and other hormones, depending on the type of hypophyseal function reduced. In the absence of the effect of gonadotropic drugs, an injection of androgens is prescribed.

Prognosis for craniopharyngioma

Prognosis is due to the timeliness of therapy and the professionalism of the doctors conducting the surgical operation.

Relapses most often develop in the first three years in patients who underwent partial removal of the tumor.

For the prevention of diabetes, the patient must be prescribed hormone replacement therapy.

Radiation therapy is effective in stopping tumor development, however, irradiation in pediatric patients causes an irreversible mental retardation and hepatic lesions. All patients after the treatment should be observed for a long time at the endocrinologist.

The video shows the removal of craniopharyngioma:

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