Among the tumors of the hypothalamic region, in addition to the craniopharyngioma, there are gliomas, hemangiomas, dysgerminomas, hamartomas, ganglion neurinomas, ependymomas, medulloblastomas, lipomas, neuroblastomas, lymphomas, plasmacytomas, colloid and dermoid cysts, sarcomas.
Depending on the location of the lesion, varying degrees of severity of neurological symptoms, impaired pituitary functions, and behavioral changes are noted. In rare cases, especially in childhood, hypothalamic lesions can lead not only to a decrease, but also to activation of adenohypophysial functions (for example, the appearance of hyperprolactin -emia due to the “removal” of the inhibitory effect of dopamine on the secretion of prolactin or premature sexual maturation due to loss of normal refractoriness to the effects of gonadotropins).
Clinic
The clinical manifestations of these lesions will depend on the age at which the tumor manifested, its location and size. The most prominent clinical manifestations are hypogonadism or premature puberty (more than 50% of cases), diabetes insipidus (up to 30% of cases), mental disorders (one third of all cases), about a third of patients — obesity or hyperphagia, in 20% of patients The main symptoms include somnolescence, anorexia, exhaustion, impaired thermoregulation, and finally, 10% disrupt the sphincter activity. Approaches to the diagnosis and treatment of these tumors are similar to those with craniopharyngiomas.
Tumor processes in the hypothalamic-hypophysial region rarely have to be differentiated from systemic and genetic lesions.
Involvement of the hypothalamus in the pathological process is possible with the disseminated specific or nonspecific infectious process, as well as with the dissemination of systemic diseases.
As a rule, a clear clinical picture of hypopituitarism with the loss of one or another function or with the development of hyperprolactinemia is formulated rather in the chronic disseminated process, while in acute bacterial injury, general systemic symptoms appear (intoxication, disruption of activity). CNS), and hypothalamic disorders are more often manifested by the syndrome of inadequate vasopressin production.
The likelihood of development of a lesion depends largely on age. In newborns, hypothalamus may suffer as a result of perinatal hemorrhage or bacterial meningitis, at the age of several months histiocytosis may develop, tuberculous meningitis may also develop in older children, may be leukemic infiltrates, as well as encephalitis. With 10 years increases the likelihood of sarcoidosis. These lesions are also possible in mature age.
Many diseases of the hypothalamus, as well as any other pathological processes in the suprasellar region, can lead to compression of the pituitary stalk with the development of the syndrome of an isolated pituitary gland. Damage to the pituitary stem is accompanied by a characteristic change in the secretion of pituitary hormones. Non-diabetes mellitus develops in 80% of patients, with the height of leg damage being the most important factor in its development: the closer the level of damage to the hypothalamus, the more likely non-sugar diabetes will develop.
In an isolated pituitary syndrome, the secretion of all tropic hormones of the pituitary gland with the development of secondary hypogonadism, hypothyroidism, hypocorticism, and growth hormone insufficiency stops. Pathognomonic for the syndrome of isolated pituitary gland phenomenon is hyperprolactinemia.
Treatment
Treatment of patients with this syndrome includes the removal of a detected tumor, replacement therapy for non-sugar diabetes and panhypopituitarism.