Syndromes in pathology of the epiphysis
Last reviewed: 19.10.2021
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Violation of puberty in humans with tumors of the epiphysis was one of the first indications of the endocrine role of this organ.
Premature sexual and somatic development occurs in congenital hypoplasia or aplasia of the pineal gland. The share of tumors of this gland accounts for less than 1% of all intracranial neoplasms. There are three types of epiphysis tumors: pinealomas (tumors originating from the parenchymal cells of the organ and occurring in 20% of its neoplasms), glial tumors (25% of epiphyseal tumors) and germinomas (the most frequent tumors of this organ). The latter are also called teratomas, and in the case of their appearance outside the pineal gland - ectopic pinealomas. They develop from germinative cells that, in the process of embryogenesis, have not reached their normal position in the sex glands and are similar in structure and function to tumors originating from the germinal cells of the ovaries and testes.
Tumors of this type usually grow into the walls of the third cerebral ventricle and into the hypothalamus, causing a characteristic triad of signs: atrophy of the optic nerves, diabetes insipidus and hypogonadism. Tumor masses can squeeze the Sylvia water pipe, resulting in internal hydrocephalus with severe headaches, vomiting, edema of the nipple of the optic nerve and a violation of consciousness. Pressure on the upper tubercles in a number of cases causes the appearance of Parino syndrome (combined paralysis of the gaze upward), and pressure on the cerebellum or brain stem is a violation of the gait. In rare cases, the hypothalamic symptoms come to the fore in the clinical picture of the disease: a change in thermoregulation, hyperphagia or anorexia. Even less often, germinoma spreads to the region of the Turkish saddle and is manifested by symptoms of tumors of the pituitary gland.
Premature puberty actually occurs only in a small percentage of cases of epiphyseal tumors, mainly with their significant spread beyond the body (Pellizzi syndrome). The fact that nonparenchymal tumors of the pineal gland are more often accompanied by premature sexual development than the parenchymal ones confirms the idea of production by the epiphysis of a factor inhibiting the production of gonadotropins and slowing down the onset of puberty. In the destruction of pinealocytes with a nonparenchymal tumor, the production of this factor probably decreases, the gonadotropic function of the pituitary gland disinhibits, and the pubertatus occurs earlier than usual. In other tumors of the epiphysis, accompanied, probably, by excessive production of this factor, on the contrary, there is a delay in puberty. However, in such cases, it has not yet been possible to detect a rise in the level of melatonin in the blood.
Symptomatic of epiphyseal tumors usually manifests late, and the clinical diagnosis is established, as a rule, at the stage when the tumor is inoperable. Radiation therapy is used, which is particularly effective in the case of the term.