Craniopharyngioma

Craniopharyngioma is a congenital brain tumor that develops from embryonic cells, the so-called Rathke's pouch.

It is usually a benign tumor that occurs at any age. Craniopharyngioma is a slow-growing tumor.

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Symptoms craniopharyngiomas

Craniopharyngioma manifests itself depending on the patient’s age:

• Children often experience dwarfism, sexual underdevelopment, and obesity due to dysfunction of the hypothalamus;
• In adults, visual acuity usually decreases and visual field defects appear.

Visual field defects arise from damage to the optic nerves, chiasm, or tracts.

• Initial defects often appear in both inferotemporal quadrants of the visual field, as the tumor compresses the chiasm superiorly and posteriorly, damaging the superior nasal fibers.
• Further, the defects spread to the superior temporal quadrants of the visual field.

The disease duration varies from several months to 10-15 years. Endocrine disorders are observed in approximately 85% of patients. With endosellar growth of craniopharyngioma, the functions of the pituitary gland are impaired, up to the development of panhypopituitarism, i.e. all tropic functions of the pituitary gland disappear, which is manifested primarily by sexual and physical underdevelopment. Rarely, with craniopharyngiomas, isolated loss of the gonadotropic function of the pituitary gland occurs, giving the clinical picture of secondary hypogonadism. Patients also experience polydipsia, impaired thermoregulation, anosmia, visual impairment and other symptoms of damage to the hypothalamic-pituitary region.

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Diagnostics craniopharyngiomas

When X-raying the skull, petrifications are detected in the area of the sella turcica or above it in 60-75% of patients.

MRI visualizes the tumor location but not calcification, which is present in 50-70% of cases. Solid tumors appear isointense on T1-weighted images. Cystic components appear hyperintense on T1-weighted images.

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

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Treatment craniopharyngiomas

Treatment of craniopharyngioma is surgical, so patients should be referred to a neurosurgeon for a treatment plan. In all cases, replacement therapy with gonadotropic (as in hypogonadism) and other hormones is prescribed in accordance with the decrease in the tropic functions of the pituitary gland. If treatment with gonadotropins is ineffective, androgens are added (injections of Sustanon-250, 1 ml once a month).

Postoperative radiotherapy may be effective, but recurrence is common, requiring lifelong monitoring.