Craniopharyngioma

Craniopharyngioma is a congenital brain tumor that develops from embryonic cells, the so-called Ratke pocket.

This is usually a benign tumor that occurs at any age. Craniopharyngioma is a slowly growing tumor.

[1], [2], [3]

Symptoms of the craniopharyngiomas

Craniopharyngioma is manifested depending on the patient's age:

• In children, dwarfism, sexual underdevelopment and obesity are often observed   due to impaired hypothalamic function;
• In adults, visual acuity usually decreases and visual field defects appear.

Defects of the visual field arise due to damage to the optic nerves, chiasma or tracts.

• Initial defects often appear in both lower-temporal quadrants of the visual field, since the tumor squeezes the chiasmus from above and behind, damaging the upper nasal fibers.
• Further defects spread to the upper temporal quadrants of the field of view.

The duration of the disease varies from several months to 10-15 years. Endocrine disorders are noted in about 85% of patients. With endosellar growth of craniopharyngioma, the functions of the pituitary gland are violated, up to the development of panhypopituitarism, ie, all tropic functions of the pituitary gland disappear, which is manifested primarily by sexual and physical underdevelopment. Occasionally, with craniopharyngiomas, there is an isolated prolapse of the gonadotropic function of the pituitary gland, giving the clinic a secondary hypogonadism. Patients also encounter polydipsia, thermoregulatory disorders, anosmia, visual impairment, and other symptoms of the hypothalamic-pituitary region.

[4], [5]

Diagnostics of the craniopharyngiomas

When radiographically examining the skull in the area of the Turkish saddle or above it, in 60-75% of patients, petrification is determined.

MRI visualizes the location of the tumor, but not calcification, which is represented in 50-70% of cases. Solid tumors on T1-weighted tomograms look iso-intensive. Cystic components on T1-weighted tomograms are seen as hyperintensive.

CT and radiography show calcification, but it is not pathognomonic for craniopharyngioma, as it occurs in other parahiazarmal lesions, such as meningiomas, aneurysms and chordomas.

[6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16],

Treatment of the craniopharyngiomas

Treatment craniopharyngioma operative, so patients should be referred to a consultation with a neurosurgeon to choose the tactics of treatment. In all cases, substitution therapy with gonadotropic (as in hypogonadism) and other hormones is prescribed in accordance with the decrease in tropic functions of the pituitary gland. If treatment with gonadotropins is ineffective, then androgens are added (injections of sustanone-250 per 1 ml once a month).

Postoperative radiotherapy may be effective, but relapses are frequent, which requires lifelong observation.