Endocrinology / / May 18, 2016
Macrogenitosomia praecox (. From the Greek makros - large Latin genitalia -.. Sexual organs and the Greek soma, -body) pineal origin in most cases develops in tumors of the pineal gland.In the literature, up to 25 different types of tumors of the pineal gland, but the most common teratoma or pinealoma.The term "pinealoma" Krabbe was proposed to refer to an embryonic tumor - teratoma, but in the future this term came to be called all the pineal gland tumor (chorionepithelioma, glioma, ganglioneuroma, hemangiomas, Kaposi et al.), Regardless of their origin and histological structure.The etiology of tumor processes of the pineal gland, as well as all tumors is unknown.
premature sexual and somatic development may lead syphiloma and teberkulomy epiphysis.
With regard to the pathogenesis of the disease, there are different views in the literature.So, Askanasy (op. At IB Havin) believes that activation of growth and sexual development during embryonic tumors of the pineal gland is not due to loss of pinea
Possibility of macrogenitosomia praecox in various inflammatory and degenerative processes in the midbrain, and often to detect changes in the hypothalamus in tumors of the pineal gland gave grounds to some authors (Zondek; Bishop) argue that premature sexual and physical development is not so much pineal as hypothalamicorigin.All clinical syndrome when this is caused by the destruction or compression of the hypothalamus, particularly centers that regulate the function of the anterior pituitary.Some authors deny completely any sort was the role of the pineal gland in the event macrogenitosomia praecox.
These contradictions in the views on the pathogenetic role of the pineal gland in the event of premature physical and sexual development suggests that there is still quite a clear idea of the complex functional relationship between the pineal gland and midbrain.When establishing these relationships can not, apparently, does not take into account the fact that the pineal gland as the pituitary gland, is an appendage of the brain and is a single system with the midbrain, the nuclei of the hypothalamic region which is currently attributed to the secretory function.Probably, the pineal gland in relation to the hypothalamus in the growing organism has an inhibitory effect on sexual and somatic growth through inhibition of pituitary function in relation to production of gonadotropin and growth hormone.Inflammatory and degenerative processes in the epiphysis or the midbrain, and likewise defeated each of them individually, can remove the "brakes" and lead to the development macrogenitosomia praecox.
Clinic.Tumors of the pineal gland are observed predominantly in males.According to Kitay and Altschule, based on an analysis of 473 cases of pineal gland tumors, described in the literature, 50% falls in the age under 20 years, and the boys of the pineal gland tumors observed 3 times more often than girls.Clinical observations show that not in all cases, even in children, accompanied by the pineal gland tumor development macrogenitosomia praecox.Thus, Bishop leads the literature concerning 56 children with a tumor of the pineal gland.Of these premature sexual and physical development was observed in only 20 boys and only one girl (some researchers have even questioned the possibility of the development of pineal syndrome in girls).
AP Burlutskiy observed signs of premature sexual and physical development only in 5 of 17 children with a tumor of the pineal gland.
Gipergenitalizm pineal origin in boys appears to increase penis size, sexual glands, early awakening sexual feelings, the appearance of erection, ejaculation (sperm found in eyyakulyate).At the same time there are secondary sexual characteristics: body hair in the pubic area, armpits, mustache growth of beard;voice changes (it becomes low, the rough).Along with symptoms gipergenitalizma also observed premature development of muscles and skeleton.Due to the high growth it is never at the pineal syndrome early closure of the epiphyseal lines.Often, there is a sharp discrepancy between the trunk and limbs length: long torso and short legs.With regard to intellectual development, it is changed in different ways: in some patients with mental growth slightly ahead of the actual age, while others - age-matched, and sometimes, especially in the early symptoms of internal hydrocephalus, mental retardation occurs.Children's mentality combined with premature sexual development is reflected in the character of the sick children, their behavior and without proper supervision at home and at school can lead to tragic consequences.
As already mentioned, the girls gipergenitalizm pineal origin observed very rarely, and in adults of both pineal gland tumor sexes generally not only accompanied by increased activity of sex glands, but often cause them hypofunction: women develop amenorrhea, and men - the weakening of libidoand involution of the testes.
As you know, premature sexual and physical development may cause different origin and histological structure of the pineal gland tumor.
macrogenitosomia praecox boy 9 years old (right), on the left - a healthy boy of the same age and a rear view.
radiograph forearm and hand of a healthy boy of 9 years
radiograph forearm and wrist of the patient macrogenitosomia praecox.Early closure of the epiphysis of metacarpal bones, phalanges and epiphyseal regions of the forearm bones.
define their character on the clinical manifestations of the disease is not always possible.In this respect, a certain value of the speed of brain tumor symptoms epiphysis.In benign tumors of the pineal gland brain symptoms develop slowly, they usually precede the endocrine (gipergenitalizm) manifestations of the disease;on the contrary, they are developing rapidly in malignant tumors.The nature of the cerebral symptoms, speed of development also depends on the direction of growth of the tumor, the degree of damage to adjacent areas of the brain, and circulatory disorders liquorodynamics.
epiphyseal tumors can simulate encephalitis, a tumor of the III ventricle, the tumor of the cerebellum and the bridge.
One of the earliest manifestations of pineal gland tumors are symptoms caused by increased intracranial pressure.It should be noted, however, that children's symptoms of increased intracranial pressure may occur later, depending on the cleft bone seams and features liquorodynamics.Increased intracranial pressure is manifested by headaches, localized in the occipital and parietal regions (sometimes pains are diffuse in nature), and vomiting.By the cerebral symptoms are stagnant and nipples.The consequence of stagnant nipples can be a secondary optic atrophy with progressive fall of view, even blindness.
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- MACROGENITOSOMIA PRAECOX.Diagnosis and differential diagnosis
Increased intracranial pressure in tumors of the pineal gland is most often develops as a result of compression of the aqueduct of Sylvius.During germination of tumor or compression of the midbrain revealed symptoms of quadrigemina: first, there is a vertical nystagmus and upward gaze palsy (Parinaud syndrome), and then develop paralysis of the eye muscles, hearing loss.Germination of the tumor in the cerebellum is usually accompanied by abnormal gait, ataxia (ataxia), the appearance of nystagmus, intention tremor, chanting speech, decreased muscle tone and tendon reflexes fading.
Sometimes pineal gland tumors develop symptoms suggestive of involvement of the hypothalamic region.It is known that hypothalamic area is connected with numerous afferent and efferent pathways with different parts of the brain, there are localized higher autonomic centers that regulate the body's metabolism, not excluded neurosecretory hypothalamic function.
observed at tumors of the pineal gland diabetes insipidus put in communication with the mechanical effect of the tumor on the hypothalamus system - neurohypophysis.The Bing, based on their own observations and literature data showed that when the pineal gland tumors are often found in the section changes in the pineal gland tissues, especially in the diencephalic region.During the life of these patients, along with other symptoms, there are polydipsia, polyphagia, polyuria, drowsiness and other symptoms characteristic of the defeat of the midbrain.Some authors emphasize the need to engage with the process of the tuber cinereum.
apparently with midbrain lesions sometimes observed to be associated with tumors of the pineal gland disorders of fat metabolism (obesity), respiration and blood pressure.In those cases where the pineal syndrome develops due to myocardial encephalitis revealed certain focal cerebral symptoms characteristic of the disease.