Pituitary adenoma children
The morphological types of adenomas in children as there are differences, mainly depending on the age group:
• Children dopubertatnogo age (before puberty), the most frequent kortikotropinomy causing pituitary Cushing's (BIC), followed by prolaktinomy, fewer (but with almost equal frequency) - somatotropinomy and hormone-inactive adenomas;
• children enter adolescence dominated prolaktinomy, then kortikotropinomy, somatotropinomy and hormonally inactive adenomas.
Pituitary Cushing's.
Occurrence of pituitary Cushing's children is significantly less than in adults. The most frequent cause of rising cortisol levels in childhood are adrenal tumors, whereas the proportion of corticotropin is about 30%. Ratio of girls to boys is 1-2:1. The vast majority of children are afflicted with pituitary Cushing's older than 9 years, patients at an earlier age are rare.
Clinical manifestations of BIC in children are typical for this disease. A characteristic feature of childhood manifestation of the disease is slowing growth up to a full stop it occurring in 65-75% of children. In mild form it may be the only symptom that leads to late diagnosis of the disease, which is similar to those of the adults with this disease.
Treatment. Treatment of pituitary Cushing apply two alternative methods: surgical (removal transsfenoidalnoe adenomas) and radiation (proton therapy).
Abroad, the method of choice for treatment of BIC is transsfenoidalnaya selective adenomectomy.
Prolaktinomy (prolactin secreting pituitary adenoma).
In children, the greatest frequency of occurrence of prolactin occurs during puberty. Girls get sick more often 3-4 times higher than boys. Most of the girls disturbed menstrual cycle in the form of failure rate, decrease or complete cessation of emissions. Less disease is manifested by headaches fronto-temporal localization.
Young boys sexual disorders occur much less frequently manifested hypogonadism * and / or gynecomastia **. Because these violations are often blamed on the delay of sexual development, a tumor diagnosed at the stage makroprolaktinom when joining neurological symptoms (headaches, visual disturbances) associated with large tumor size.
In addition to this may be a delay of growth and / or overweight.
Treatment of prolactin.
Currently, there are two ways of treatment: surgical removal and the use of dopamine agonists (DA). As surgical treatment is applied transsfenoidalnaya (transcranial or - very rarely) adenomectomy. The choice of surgical intervention depends on the size, growth direction and extent of the tumor.
However, to date method of choice for most authors consider the use of dopamine agonists. With good sensitivity to the drug even when a large amount of tumor reduction in tumor mass, and improvement may occur in the shortest period of time from several days to 1-2 weeks of starting treatment. However, the choice of treatment is always for specialists.
Somatotropinomy.
Somatotropinomy in children occur at approximately 9%. Most often they are diagnosed in children 12-15 years. Clinical manifestations of this type of adenomas in children are bright enough: marked acceleration in growth, resulting in gigantism, a third of patients are observed akromegaloidnye change and a girls' puberty illness accompanied by disturbances in the menstrual cycle.
Most of the tumor reaches a considerable size, which is accompanied by appropriate neurological symptoms as headaches, visual disturbances.
Treatment somatotropinomy.
Currently, most researchers tend to Neurosurgical removal of the tumor. Given the fact that about half the children have somatotropinoma invasive growth, the choice of access is particularly important. Used transsfenoidalnoe or transcranial removal of adenomas. In addition to the neurosurgical treatment of dopamine agonist use (Bromcriptin, Cabergoline, Dostinex, etc.). The effective use of long-acting somatostatin analogues.
Thus, when the appearance of children above symptoms should immediately contact a specialist neurosurgeon or endocrinologist.
Should undertake a comprehensive hormonal examination to determine the type of hormonal activity of the tumor, as well as radiography of the skull, a computer and / or MRI of the brain, and then choose the most appropriate method of treatment.
After treatment for pituitary adenoma should be prolonged observation of specialists to provide timely detection of possible recurrence of the tumor, as well as the selection of hormone replacement therapy.
Adequate, timely replacement therapy can significantly improve the condition and quality of life of patients treated at the pituitary adenomas in childhood.
hypoplasia hypogonadism * genital organs and secondary sexual characteristics in relation to reduced secretion of sex hormones;
gynecomastia ** one-or two-sided increase in male mammary glands, sometimes by the type of female.
Bibliography:
1. NA Strebkova, AN Tyulpakov, SK Gorelyshev, VA Peterkova "Clinical manifestations, diagnosis and treatment of pituitary adenomas in children", the materials of the Russian scientific-practical conference, Moscow, 2001.
2. Copinschi G., Neve P., Walter R., Bastenie P.A. Dwarfism caused by Cushing's syndrome. Report of a case / / Acta Endocrinol (Copenh). - 1969. - 60:446-450.
3. Dominque J.N., Richmond I.L., Wilson C.B. Results of surgery in 114 patients with prolactin-secreting pituitary adenomas / / Am J Obstet Gynecol. - 1980. - 137:102-108.
4. B. Fraioli, Ferranle L., Celli P. Pituitary adenomas with onset during puberty: Features and treatment / / J Neurosurg. - 1983. - 59:590-95.
5. HaddadS.F., VanGilderJ.C., Menezes AH: Pediatric pituitary tumors / / J Neurosurg. - 1991. - Vol.29. - No. 4:509-514.
6. Levy S.R., Wynne C.V., Lorentz W.B. Cushing's syndrome in infancy secondary to pituitary adenoma / / Am J Dis Child. - 1982. - 136:605-7.
7. Ludecke O.K., Hermann H.D., Schulte FJ. Special problems with neurosurgical treatment of hormone-secreting pituitary adenomas in children / / Prog Exp Tumor Res. - 1987. - 30:362-370.
8. McArthur RG, Gloutier MD, Hayles AB, Sprague RG Cushing's disease in children. Findings in 134 cases / / Mayoclin Proc. - 1972.-47 :318-326.
9. Melmed S. Acromegaly. In: Melmed S., ed. The pituitary / / Cambridge: Blackwell. - 1995. - 413-442. Wass J.A.H. Octreotide treatment of acromegaly / / Horm Res. -1990.-l (suppl): l-5.
10. Mindermann T., Wilson C.B. Pediatric pituitary adenomas / / J Neurosurg. - 1995. - Vol. 36. - No. 2:259-269.
11. Van Gilder J.C. The hypothalamic anterior pituitary relation ships and their tumours. A review / / Surg Neurol. - 1988. - 30:187-96. 38.
Keywords page:
pituitary, adenoma, children, BIC, Treatment, Cushing, prolactin secreting, neurological symptomsAnnouncement articles
Hormones- secretion, delivery of hormones to target cells and the metabolismHormones-secretion, a general description
Cushing's Syndrome: Diagnosis
Primary Adrenal insufficiency (Addison disease)
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