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Pituitary-adrenal axis in pinealoma.

Autopsy series of 19 patients with pineal and anterior third ventricle (suprasellar) tumors were analyzed with regard to shift in pituitary-adrenal axis. Germinomas formed the most common subgroup in this series (79%). Metastatic tumor growth in the pituitary gland caused adrenal atrophy in 4 cases. Some patients with pineal neoplasms had larger adrenals in weight compared with age- and sex-matched controls of Japanese. In three patients with sexual precocity, adrenal weight was larger. It is concluded that clinical importance exists in anterior pituitary insufficiency with adrenal atrophy, but hyperfunction of the hypophyseoadrenal system might occur during the course of the disease.

Adolescent↗

Parental origin of Gsalpha mutations in the McCune-Albright syndrome and in isolated endocrine tumors.

Activating mutations of the Gsalpha gene are detected in different endocrine tumors, such as GH-secreting adenomas and toxic thyroid adenomas, and in hyperfunctioning glands from patients with McCune-Albright syndrome (MAS). There is increasing evidence that the Gsalpha gene is subjected to imprinting control and that Gsalpha imprinting plays a key role in the pathogenesis of different human diseases. The aim of this study was to investigate the presence of a parent specificity of Gsalpha mutations in 10 patients affected with MAS and 12 isolated tumors (10 GH-secreting adenomas, one toxic thyroid adenoma, and one hyperfunctioning adrenal adenoma). The parental origin of Gsalpha mutations was assessed by evaluating NESP55 and exon 1A transcripts, which are monoallelically expressed from the maternal and paternal alleles, respectively. By this approach, we demonstrated that in isolated GH-secreting adenomas, as well as in MAS patients with acromegaly, Gsalpha mutations were on the maternal allele. By contrast, the involvement of other endocrine organs in MAS patients was not associated with a particular parent specificity, as precocious puberty and hyperthyroidism were present in patients with mutations on either the maternal or the paternal allele. Moreover, isolated hyperfunctioning thyroid and adrenal adenomas displayed the mutation on the maternal and paternal alleles, respectively. These data confirm the importance of Gsalpha imprinting in the pituitary gland and point out the high degree of tissue specificity of this phenomenon.

Adenoma↗

The hypothalamo-hypophysial system of the lemming, Dicrostonyx torquatus Pallas. III. Population aspects of neuroendocrine regulation.

The structure and ultrastructure of the following regions of the hypothalamo-hypophysial neurosecretory system (HHNS) in the population cycle of lemming were studied: supraoptic (SON), paraventricular (PVN) and arcuate nuclei (AN), the median eminence (ME) and posterior pituitary (PP). Being compared to the state of endocrine glands and gonads, and the level of reproduction stress in the population cycle of the lemming described earlier, the obtained data revealed a certain regularity. First, at the stages of decrease, increase and in the first period of the stage of population peak the activity of the SON-PP complex and AN is gradually increased. This coincides with intensified reproduction, intensified functioning of the thyroid gland and adrenal cortex. Second, while the population is decreasing, all the above functions become depressed, except that of the thyroid gland which, on the contrary, becomes active. In the second period of the stage of population peak, when reproduction stops abruptly, this regularity, however, does not hold, the activity of SON-PP complex and AN is sharply lowered, whereas that of PVN becomes very high. The structure of the thyroid damaged as a result of previous hyperfunction, is restored, and the adrenal cortex shows signs of hypercorticism. The present work is devoted to specificity of the HHNS functioning at the stage of population peak, referred to as crucial, and the pathways and mechanisms by which neurohormones produced by this system can affect the endocrine glands. In addition, the causes likely to lead to the mass death of animals at this stage, and biological significance of this fact are also given attention to.

Animals↗

Serum corticosterone concentrations in reproductively mature and inhibited deermice (Peromyscus maniculatus bairdii).

Serum corticosterone concentrations were measured to see if there was any correlation between adrenal activity and reproductive inhibition. Males and females from two growing and one stable (asymptotic) population had significantly smaller reproductive organs (seminal vesicles, testes, uteri, ovaries) than controls. The weights of the adrenal glands of population mice were smaller, often significantly so, than those of controls, but the mean serum corticosterone concentrations were higher than those of control mice for the two growing populations and significantly higher for the asymptotic population. These data suggest that adrenal function may be associated with the reproductively inhibited condition, but not in a direct or dose-response fashion and that adrenal hyperfunction in this species may not be reflected by adrenal hypertrophy.

Adrenal Glands↗

[Ectopic ACTH syndrome (author's transl)].

Ectopic ACTH syndrome is a clinicopathologic condition produced by certain tumors which release hormone that is indistinguishable from ACTH. It orginates the chemical and clinical anomalies characteristic of Cushing's syndrome by its action on the adrenal glands. The tumors may be present in any organ, though they are most frequently found in the lungs, thymus, pancreas or gastrointestinal tract. They may be benign or malignant, though usually the latter. Secretion of the hormone is completely autnomous; it is release in a way similar to that of the hypophysis. Not infrequently other hormones besides ACTH are also produced, such as MSH, serotonin, and CRF. Ectopic ACTH is of higher molecular weight than hypophyseal ACTH, which suggest it may be comprised of the latter bounded covalently to a peptide. The clinical course is rapid, so that not all of the symptoms of Cushing's syndrome develop. Moon face, osteoporosis, and obesity are typically lacking; melanodermia and hypokalemic alkalosis ofter appear. Laboratory data include an increase in ACTH and cholesterol concentrations, disappearance of the nictameral rhythm, and an increase in urinary excretion of 17-hydroxycorticoids and 17-ketosteroids. Stimulation and supression tests are abnormal. The prognosis is poor and the only possible treatment is a complete surgical removal of the tumor. Irradiation or chemotherapy could be applied as well as the correction of the adrenal hyperfunction by the administration of drugs or by total bilateral adrenalectomy.

Adrenal Glands↗

Primary adrenocortical nodular dysplasia presented as adrenal adenoma by functional scintigraphy.

This is a report of a case of a 12-year-old boy with Cushing's syndrome in which high doses of dexamethasone failed to suppress glucocorticoid secretion. Neither CT nor MRI (morphologic studies) revealed any adrenal abnormality. Functional images with NP-59 (I-131 beta-iodomethyl-norcholesterol) clearly revealed intense unilateral uptake in the right adrenal gland. A right adrenalectomy was performed, and the pathologic diagnosis was primary adrenocortical nodular dysplasia. One year later, the cushingoid signs had virtually disappeared and urinary steroid excretion and serum cortisol levels were within the normal range. Nevertheless, primary adrenocortical nodular dysplasia is essentially a bilateral disease. In this case, a remission in the adrenocortical hyperfunction was observed. Although there was confusion in the etiological diagnosis, functional scintigraphy correctly indicated the status of adrenal function.

Adenoma↗

[Multiple endocrine adenomatosis manifested chiefly by hyperparathyroidism].

A case with multiple endocrine neoplasia was reported, including parathyroid adenoma, of the main cells of the gland, multiple small adenomas in the tail of pancreas, (cytologically and electron-microscopically determined as A cellular) and light-cellular adenoma of adrenal. The hyperfunction of parathyroid adenoma was manifested with hypercalcemia (3.75 mmol/l), and morphologically--with the multiple calcium metastases in lungs, kidneys and heart, established at necropsy. The cause for the death was the acutely advanced ischemic disease of myocardium, on the background of chronic pyelonephritis and renal insufficiency. The timely diagnosis of such morbid states is concluded to be important for the clinical practice and could lead to the saving of the patients by operative removal of the tumour.

Adenoma↗