Hypothalamic-hypophysial diseases can be subdivided into diseases with a proven lesion of the hypothalamus proper, a disease with a hypothetical hypothalamic genesis, with hypothalamic-hypophysial genesis and the hypopharyngeal lesions proper.
Craniopharyngioma
Craniopharyngioma is a hypothalamic tumor, originating from the remnants of Rathke’s pocket (epithelial protrusion of the posterior pharyngeal wall of the embryo, which is the germ of the adenog-pofiz), leading to pituitary disorders.
Pathogenesis
Tumor development is associated with impaired embryonic differentiation of Ratke’s pocket cells. The tumor may be localized in the hypothalamus, the third ventricle, the Turkish saddle, and more often has a cystic structure. Craniopharyngioma is a rare disease, but the most frequent suprasellar tumor in children (5–10% of brain tumors in children).
Craniopharyngiomas are hormonally inactive tumors, at the basis of the clinical manifestations of which there is a mechanical compression of the surrounding structures of the brain.
Clinic
In most cases, craniopharyngioma manifests in childhood and adolescence. As a rule, there is a combination of symptoms of intracranial hypertension (headache, nausea, vomiting), chiasmatic syndrome (bitemporal hemianopsy, optic nerve edema, decreased visual acuity) and endocrine-exchange syndrome (delayed sexual and physical development, hypopituitarism). The development of cerebral edema or panhypopituitary coma is an indication for emergency hospitalization.
Diagnostics
A hormonal study determines the deficiency of the tropic hormones of the pituitary gland; hyperprolactinemia is possible. Radiologically, in 80% of cases, calcifications are detected in the tumor. MRI is the method of imaging diagnosis of craniopharyngiomas.
Craniopharyngiomas should be differentiated with other diseases occurring with delayed sexual and physical development and hypopituitarism, as well as with other tumors of the pituitary and brain.
Treatment
Treatment is indicated by surgical removal of the tumor, possibly in combination with proton therapy and stereotactic introduction of radioisotopes into the tumor. When incomplete removal of the cranio-pharyngioma tends to recur. Restoration of the childbearing function after removal of the craniopharyngioma with the help of modern methods of treatment is possible in principle. The prognosis for life with a craniopharyngioma is quite serious, since surgical treatment does not eliminate the metabolic endocrine disorders, and the patient’s ability to work is always limited. With the development of hypopituitarism, reparative therapy is carried out for life.