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Evidence of abnormal dopaminergic control of prolactin in patients with hypothalamic and pituitary tumors.

Prolactin secretion was investigated in an attempt to identify the patterns of responses in different types of tumors. Forty four patients were studied: thirty patients with prolactinomas (Group 2); nine patients with growth-hormone (GH)-adrenocorticotropic hormone (ACTH)-secreting pituitary tumors and hypothalamic tumors (Group 3); and five patients with non-secreting pituitary tumors (Group 4). A control group (Group 1) consisted of 60 healthy subjects (30 males and 30 females). All were submitted to testing by nomifensine (Nom), domperidone (Dom) and thyrotropin releasing hormone (TRH). The prolactin levels were measured by radioimmunoassay (RIA). In group 2 the suppression of PRL with Nom and the stimulation with Dom and TRH were significantly lower than in the control group (p less than 0.001). There was no statistically significant difference between groups 2 and 3 in the suppression with Nom. The increase with Dom in group 3 was significantly greater than that in group 2 (p less than 0.001) and less than that in the control group (p less than 0.005). The rise in PRL with TRH was also significantly higher in group 3 than in group 2 (p less than 0.001) and similar to that of the control group. Group 4 gave the same results as the control group to all 3 tests. Our results indicate a dopaminergic irregularity in the hypothalamic and GH-ACTH-secreting pituitary tumors, thus supporting a hypothalamic etiopathogenesis of these tumors. The normality of the GH-ACTH-secreting pituitary tumors and hypothalamic tumor responses to TRH is one more factor in differentiating these from prolactinomas. The normal response of the non-secreting tumors may involve a primary pituitary etiology of these tumors.

Adrenocorticotropic Hormone↗

[Thyrotropic function in patients with a hypothalamic or pituitary tumor. Possibility of the secretion of a TSH with reduced biological activity].

Thyrotropic function was studied in 100 patients with hypothalamic or pituitary tumours before treatment. Eighteen patients with thyroid deficiency showed no signs of primary hypothyroidism. This was also showed in 4 other hypothyroid patients who had pituitary tumours studied later on. Only 4 of these 22 patients had TSH deficiency. The other 18 had normal or high plasma TSH levels, and the TSH response to TRH was normal but often delayed and/or prolonged. This pattern, suggestive of secretion of TSH with reduced biological activity, might however be due to other factors. Tumoral invasion of the hypothalamus, present in these 18 cases, could reduce secretion of TRH and dopamine. Hypothyroidism would then be secondary to TRH deficiency if TRH is considered to have a direct thyroid-stimulating action, and, like dopamine deficiency, could contribute to maintaining normal or high TSH secretion.

Adenoma↗

[Changes in the dendrite ultrastructure of the cerebral cortex neurons in human tumors located in the hypothalamo-hypophyseal area].

By means of electron microscopy method of bioptic material structure of the neuronal islets of the cerebral cortex has been studied in 5 persons with benign tumors that immediately effect the hypothalamus. Certain changes in ultrastructure of dendrites are revealed according to the light and dark types, as well as axons, degenerating according to the dark type. The greatest changes, including degeneration according to the dark type, undergo small branches of the dendrites. Similar pictures reflect, evidently, reduction of the dendritic tree in slightly changed cortical neurons, connected with breaking of trophic influences in the hypothalamus, evoked in it by the tumor.

Adenoma↗

[Ultrastructure and morphometric characteristics of synapses of the cerebral cortex in patients with tumors located in the hypothalamo-hypophyseal system].

By means of the electron microscopy method using biopsies structure of the cerebral cortex synapses has been investigated in 4 patients with benign tumors, that immediately influence the hypothalamus. The ultrastructure is described and the data on quantitative correlation of unchanged interneuronal contacts and synapses with various manifestation of the structural disturbances are presented. The unchanged synapses make 72%, with an increased amount of the synaptic vesicles-16%, with agglutination of the synaptic vesicles-2%, with destruction of the presynaptic element after the dark type-4%, with other structural changes-6%. Results of the morphometric analysis demonstrate a decreasing functional activity of the interneuronal contacts with some pathological signs.

Adenoma↗

[Magnetic resonance imaging of hypothalamic hamartoma].

Magnetic resonance (MR) findings of two patients with a hypothalamic hamartoma are discussed. The two girls showed clinical symptoms and endocrinological signs of precocious puberty. MR imaging was of diagnostic value superior to that of CT in the demonstration of the characteristic location of this tumor and relationships to the neighboring structures because of its multi-dimensional utility. Although it has been reported that CT showed this lesion as isodense to the grey matter with and without injection of contrast medium, MR imaging depicted the lesion as a high signal intensity area on T 2-weighted images in both patients. MR imaging is a useful method for the evaluation of the hypothalamic hamartoma.

Child↗

[Controlling precocious puberty--surgical excision of hypothalamic hamartoma causing precocious puberty].

Among the causes of precocious puberty, hypothalamic hamartoma comprises a small percentage. However, the frequency of precocious puberty in the presence of hypothalamic hamartoma is quite high. Recently, results of surgery in 14 cases of hypothalamic hamartoma were reported. Precocious puberty completely subsided in three cases and slight improvement was achieved in another three cases. We performed surgery in four patients with hypothalamic hamartomas, with the goal of decreasing the symptoms of precocious puberty. The patients were two females (aged 1 yr, 3 mo and 6 mo) and two males (aged 3 yr, 7 mo and 1 yr, 9 mo). The main symptoms were precocious puberty and mental retardation of varying degrees. The males had excessive growth of body and external genitalia, while the females had genital bleeding and premature breast development. In each case, computed tomographic scans disclosed a round, isodense mass in the interpeduncular cistern, attached to the base of the hypothalamus. Contrast enhancement was negative. Endocrinologically, in case 1, testosterone was 92.6 ng/ml, FSH was 16 mIU/ml, and LH was 2.2 mIU/ml. Although LH was within normal limits, it overresponded to LH-RH stimulation. In case 2, estrogen was 13.5 ng/day, LH was 5.2 mIU/ml, FSH was 5.3 mIU/ml, and LH showed an exaggerated response to LH-RH stimulation. In case 3, testosterone was 362 ng/ml, LH was 8.8 mIU/ml, FSH was 4.8 mIU/ml, and LH showed an abnormally high response to LH-RH stimulation. In case 4, LH was 18.4 mIU/ml, FSH was 12.0 mIU/ml, and both hormones were stimulated abnormally strongly by LH-RH.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

[Primary hypothalamic tumors (excluding craniopharyngioma). Endocrine and post-radiotherapy development study. Apropos of 17 cases].

The authors underline a number of features of primary hypothalamic tumours (except craniopharyngioma) based on a study of 17 cases: The vital prognosis is satisfactory; only one death was observed with an average follow-up period of 80 months (range 1 to 17 years). This raises the question of the necessity for systematic biopsy to confirm the tumour histology (although when performed in conditions of stereotaxis there are no serious risks). The site of the tumour makes surgery difficult and complete ablation impossible. As many hypothalamic tumours, including the pilocytic astrocytoma which is the commonest type, are sensitive to radiotherapy, this would seem to be the treatment of choice. Total regression is observed in the majority of cases. From the endocrine point of view, the mediocre prognosis is aggravated by high energy radiotherapy. However, with the present therapeutic methods, it is relatively easy to compensate complete or dissociated deficits. There is an endocrine syndrome suggestive of hypothalamic tumour which consists of anterior pituitary deficiency, diabetes insipidus and moderate hyperprolactinaemia (20 to 100 ng/ml). Close medical surveillance is essential, both of the local (visual field and frontal CT scanning) and endocrine effects. Hormonal deficits may occur as a late complication up to 5 years after radiotherapy. The problems inherent to hypothalamic tumours: neurogenic hypernatraemia which is luckily uncommon, with an unexplained weight gain occurring during substitute therapy for adrenal insufficiency with physiological doses of hydrocortisone. Finally, the overall prognosis is good with a minimum of complications due to radiotherapy, excepting endocrine deficiency, providing a dose of 60 grays in 6 weeks at 2 grays per session is not exceeded.

Adolescent↗