[Ectopic ACTH syndrome].
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Biomedical subjects
Publications and source records attributed to J Załuska.
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Ten patients with Nelson's syndrome, nine women and one man, aged 22 to 61 years, were treated neurosurgically by a transsphenoidal approach. In four patients, microadenomas were found, ranging in diameter from 4 to 10 mm. Microscopically, mixed adenoma was diagnosed in six cases, basophilic adenoma in three patients, and chromophobe adenoma in one patient. The presence of argyrophilic nerve fibers in the adenoma tissue was noted in one case. The time of observation after transsphenoidal surgery ranged from 6 months to 10 years. Clinical remission was achieved in eight patients; in two of them, radiation therapy was used to complement surgical treatment. In two patients, recurrence of the pituitary neoplasm was observed; anaplasia was revealed at the second operation in one of them. Radiation therapy might be a useful adjunct to neurosurgery in Nelson's syndrome, especially in patients with anaplastic adenoma.
Fifty patients bilaterally adrenalectomized for Cushing's disease were followed for 1 to 22 (mean, 12) years. In 14 of them (28%) Nelson's syndrome appeared within 1.5 to 12 (mean, 4.8) years after adrenalectomy. All the patients were deeply pigmented, 12 had a radiologically demonstrable tumour and six had visual defects. Plasma ACTH levels ranged from 450 to 8000 ng/l. However in every case at least one estimation during circadian studies equalled 2000 ng/l. One patient with an anaplastic pituitary tumour died 3 years after the discovery of the tumour. Anaplasia was also diagnosed in another patient with recurrence of pituitary tumour after a transsphenoidal operation. Symptoms of tumour infarction occurred in two patients, followed by clinical remission of Nelson's syndrome. In the majority of cases the course was benign. We conclude that all cases, however, should be followed indefinitely because Nelson's tumours are not infrequently aggressive.
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The paper sums up the experience of the clinic in diagnosing and therapy of the Cushing syndrome due to multiple autonomous adrenal cortical adenomas. The observation was carried out in a group of 5 women between 15-44 years of age (31.7 on an average). In the clinical material including 225 cases of the Cushing syndrome, they amounted to 2.2 per cent. All the five patients had typical somatic symptoms, myasthenia, ++amenorrhea. In hormonal examinations the content of 17-OHCS in the 24-hour urine was only periodically increased, 17-KS was within the normal range. There was no inhibiting action of dexamethasone on the excretion of cortisol, even when the initial values of corticosteroids++ were normal. The concentration of ACTH in the blood (determined in 3 cases) was around low normal range. Three patients underwent computer tomography; in one case adrenal glands were of normal size, in the second case--the two glands were enlarged, in the third case--the right gland was much larger than the left one. Microscopic examinations showed the atrophy of adrenal cortex beyond the adenomas. In case of multiple adenomas of the left gland surgery was confined to unilateral adrenalectomy. Among the remaining 4 patients, 3 underwent simultaneous resection of the two glands. All the patients showed the regression of symptoms of the disease, except for the changes in bones. One patient did not turn up for further therapy after unilateral adrenalectomy because of partial improvement.
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