Kraniofaringioma is good-quality cystous the new growth which is localized in the field of the Turkish saddle which develops from the remains of an epithelium of a pocket of Ratke. In process of progressing and growth of a tumor at the patient increase in intra cranial pressure, sight violation, lag in physical and mental development, hypofunction of an adenogipofiz is observed. By the most informative methods of diagnostics of a kraniofaringioma are MPT and KT which helps to define structure, borders of a tumor and existence of kaltsinat. Treatment – surgical after which to the patient appoint a course of the radiation therapy directed to a stop of further growth of a new growth.
Kraniofaringioma is a tumor of a brain of good-quality character which comes from the embryonic period and, as a rule, settles down in gipotalamo-hypophysial area. In process of progressing of a new growth in its fabrics the cysts filled with liquid with high content of cholesterol and proteins can be formed. The disease is more inherent for children, however can develop at any age.
Development frequency kraniofaringiy makes about 2-3% of all types of new growths of a brain. Most often the neyroepitelialny kraniofaringioma (about 10% of cases) meets, and the peak of development of a disease is necessary aged from 5 up to 15 years. Other kind of a kraniofaringioma – papillary, occurs most often at people after 40 years.
Reasons of development of a kraniofaringioma
Heredity and different mutations are the main reasons for development of a kraniofaringioma. Besides, adverse factors, especially in the period of laying of the main bodies - the first trimester of pregnancy exert impact on development of pathology. Influence of medicines, toxins, poisons, radiation and so forth belongs to causal factors of emergence of a kraniofaringioma; pre-natal infection, hard proceeding early toxicosis, chronic diseases of the pregnant woman (tuberculosis, diabetes, a renal failure).
Kraniofaringioma is subdivided into 2 look: adamantinomatozny and papillary. About 60% kraniofaringiy represent cystous education, about 15% are solid educations, other 35% have the mixed structure and a structure.
Kraniofaringioma develops from the embryonic remains of a pocket of Ratke – structure from which is formed and develops subsequently. The new growth in most cases has cysts, and at adults contains kaltsifikata. Because of the localization and progressing of a kraniofaringiom causes lag in development in children, violation of visual function, a disease of bodies of endocrine system in adults.
Kraniofaringioma can clinically not be shown throughout the long period of time in any way. Most often the first symptoms of a disease arise closer by 5-10 years. Primary manifestations which force the patient to see a doctor are the constant headaches which are not stopped analgetics, disorders of neurologic character, violation in work of bodies of endocrine system.
In process of progressing of a tumor it begins to squeeze a hypothalamus, a hypophysis and a visual recross that does clinical symptomatology more expressed and bright. The most typical clinical manifestations of a kraniofaringioma are obesity and low growth.
Progressing and growth of a tumor causes deterioration in sight of the patient at the initial stage what doctors pay attention at primary survey of the patient to. On later terms of development of a disease survey of the ophthalmologist with carrying out an oftalmoskopiya finds irreversible changes at an eye bottom, hypostasis and an atrophy of an optic nerve. Expressiveness of a clinical picture in many respects depends on the size and extent of progressing of a new growth.
The main sign of a kraniofaringioma which is defined only by a radiological research of a skull is change of the Turkish saddle. In a picture thinning of its back and wedge-shaped shoots is accurately visible.
Diagnostics of a kraniofaringioma
Diagnostics of a kraniofaringioma consists in performing tool modern diagnostics and consultation of the neurologist. The X-ray analysis of a skull allows to reveal kaltsinata in tumors, an erosion of its walls and increase in the sizes of the Turkish saddle. Without fail to the patient carry out a number of analyses on identification of amount of hormones to blood (a research of the SMT, AKTG, TTG level, cortisol and hormones of a thyroid gland).
By the most informative and exact methods of diagnostics of a kraniofaringioma are brain MPT and KT. By means of these methods of a research the expert manages to receive the layer-by-layer image of brain fabrics that allows to define most precisely the place of localization of pathological process, the amount of education, structure and a tumor form, its arrangement of rather surrounding fabrics.
Kraniofaringiy needs to differentiate with other tumors of hypophysial area: hypophysis adenoma, a glioma of a hiazma, germinomy, and also with a colloidal cyst of the III ventricle. Exact differentiation of tumors is possible only after carrying out a histologic research of their fabrics.
Treatment of a kraniofaringioma
Treatment of a kraniofaringioma is carried out by a surgical way and performed by neurosurgeons. The way of access to a tumor in many respects depends on the place of its localization and the sizes. If full removal of a new growth for any reasons is impossible, then in the postoperative period use methods of radiation therapy.
The resection of a kraniofaringioma eliminates the tumor pressure upon a hypophysis and the next tissues of a brain, and by means of radiation therapy it is possible to contain the progressing growth of a tumor in 90% of cases. A modern and effective method of treatment of a kraniofaringioma is drainage of a cyst and introduction to her cavity of the antibiotic which is perniciously operating on fabrics and cells of a tumor (bleomycin).
Forecast and prevention
The forecast in most cases depends on timeliness of diagnosis and experience of doctors during performing surgery. The recurrence of a disease meets in the first 3 years after operation and arises, as a rule, during not radical removal of a new growth. One of complications in the postoperative period is development of diabetes of not sugar type therefore the patient needs long replacement therapy by hormonal medicines under observation of the endocrinologist.
Besides, application of radiation therapy though is an effective way of a stop of further growth and progressing of a tumor, but causes lag in intellectual development (ZPR) in children and severe damages of internals, in particular a liver. Prevention of pathological process consists in antenatalny protection of a fruit.