1/10. Triple pituitary adenoma in Cushing's disease: case report.A case of a triple pituitary adenoma identified in a surgically removed pituitary gland from a 52-year-old woman operated on for Cushing's disease is presented. The histology revealed 3 distinctly separate microadenomas, 1 corticotroph and 2 immunoreactive for prolactin (PRL). The latter were apparently silent, since the serum PRL levels were within normal range. The problems associated with the inability to identify multiple adenomas pre-operatively and the possible failure of selective transsphenoidal adenomectomy in case of multiple adenomas are emphasised.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/10. An R201H activating mutation of the GNAS1 (Gsalpha) gene in a corticotroph pituitary adenoma.In the pituitary gland, activating mutations of the GNAS1 (Gsalpha) gene at Gln227 have been identified in adrenocorticotrophin secreting, growth hormone secreting, and prolactin secreting adenomas. To date, mutations at the codon encoding R201, typically underlying the McCune-Albright syndrome and isolated fibrous dysplasia of bone, have been demonstrated only in growth hormone secreting pituitary adenomas. In this study, a polymerase chain reaction amplified target sequence in exon 8 of the GNAS1 gene was sequenced, identifying the first R201 mutation seen in an isolated basophilic adenoma which generated Cushing's disease in a child. This case adds Cushing's disease to the range of human diseases caused by R201 mutations of the GNAS1 gene.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/10. Cushing's disease in a 5-month infant due to a basophilic microadenoma of the pituitary gland.We report a female patient who developed severe Cushing's disease during the fifth month of life due to a basophilic pituitary adenoma Histological findings showed a basophilic microadenoma of the pituitary gland, leading to the diagnosis of Cushing's disease. The infant died because of untreatable septic shock. The importance of the present report resides in the age of the child at diagnosis, and that it was the necropsy finding of microadenoma which clarified the cause of the Cushing's syndrome, since it was not diagnosed during life. Cushing's disease is most often diagnosed in children older than 7 years, and our patient was only 5 months old when we detected the pituitary adenoma, the earliest case diagnosed so far. Cushing's syndrome in pediatric patients has been rarely reported and most cases are due to functioning adrenal tumors, usually a malignant carcinoma but occasionally a benign adenoma. The present case shows that the pituitary of these patients should be investigated with important implications in terms of therapeutic approaches, such as pituitary radiotherapy, which can cure the patient when treatment is started very soon.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
4/10. Cure of Cushing's disease by transsphenoidal removal of a microadenoma from a pituitary gland despite a radiographically normal sella turcica.A 37-year-old woman had symptoms of Cushing's disease for two years. galactorrhea was present. The diagnosis was confirmed by finding intermittently elevated urinary 17-hydroxysteroids, absent circadian rhythm, and elevated plasma ACTH. plasma prolactin was slightly elevated. Films of the sella turcica were normal. A 9 mm basophilic microadenoma was removed by the transphenoidal approach. Immunocytochemical and electron-microscopic studies showed that the tumor was composed exclusively of ACTH secreting cells. Endocrine re-evaluation one year later revealed normal adrenal function. serum prolactin had returned to normal. This case provides further evidence that Cushing's disease can be caused by a pituitary microadenoma insufficient in size to deform the sella.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
5/10. Cushing's syndrome, nodular adrenal hyperplasia and virilizing carcinoma.A 48-year-old hypertensive diabetic woman rapidly became virilized. urine 17-oxo-and oxogenic steroids and plasma testosterone, androstenedione, DHEA, DHEA-sulphate and androstenediol were greatly elevated. plasma cortisol was constantly high and was not suppressed by dexamethasone. Circulating immunoreactive ACTH was consistently detectable at 18-24 ng/l. A 450 g carcinoma arising from a nodular hyperplastic right adrenal gland was resected. Production by the tumour of 17a-hydroxypregnenolone, 17a-hydroxyprogesterone and five C-19 steroids, but very little prenenolone, progesterone or cortisol, was shown by blood sampling, tumour culture and dramatic falls after operation. The plasma cortisol fell to half, with no diurnal variation, consistent with persistent Cushing's syndrome, and the plasma ACTH rose to 55 ng/l. She died 3 months later from a myocardial infarction. autopsy revealed a pituitary basophil adenoma at a site where radiologically there had been an indentation in the fossa floor for at least 7 years. The left adrenal gland showed nodular hyperplasia. Therefore we conclude that mild pituitary-dependent Cushing's syndrome may have been present for many years before development of a virilizing carcinoma. This case demonstrates that adrenal carcinoma in man can sometimes develop as a consequence of nodular adrenal hyperplasia which may in turn be due to long-standing trophic hyper-stimulation.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
6/10. Cushing's disease in a young woman with anorexia nervosa: pathophysiological implications.This report describes a 17-year old student who was found to have Cushing's syndrome two years after she had developed anorexia nervosa (AN). The Cushing's syndrome was treated with bilateral resection of enlarged, hyperplastic, non-tumorous adrenal glands. The diagnosis was further confirmed four years later when, two to three years after new symptoms had appeared, an ACTH secreting pituitary adenoma (that is, Cushing's disease) was found on surgery. The possible mechanism for the development of Cushing's disease in a patient with prior anorexia nervosa, a sequence of events reported once previously, is discussed. It is suggested that increased hypothalamic-pituitary corticotroph stimulation in association with the anorexia nervosa, a now well-established endocrine phenomenon, activated an occult, inactive pituitary basophil adenoma in this patient, eventually resulting in autonomous pituitary overproduction of ACTH by the tumor.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/10. Silent corticotropic adenomas of the human pituitary gland: a histologic, immunocytologic, and ultrastructural study.Among 300 surgically removed pituitary adenomas, 17 tumors containing immunoreactive 1-39 adrenocorticotropin (ACTH) and/or 19-39 ACTH, beta-lipotropin, and alpha-endorphin but unassociated with clinical signs of Cushing's disease have been detected. These neoplasms were divided into basophilic adenomas with strong periodic acid-Schiff (PAS) and lead-hematoxylin positivity and chromophobic tumors with moderate or no PAS and lead-hematoxylin positivity. The former were densely granulated tumors with a fine structure strikingly similar to that of functioning corticotropic cell adenomas. The latter were sparsely granulated with varying ultrastructural patterns. The marked morphologic diversity suggests that these adenomas, despite their similar immunocytologic characteristics, represent more than one entity. Clinically, the most common finding was a rapidly progressing visual defect. An unusually high incidence of infarction (5 cases) and recurrence (5 cases) was noted, underlining the importance of correct morphologic diagnosis and careful follow-up.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
8/10. cyproheptadine-induced remission of Cushing's disease due to pituitary basophil adenoma.serotonin is involved in the control of ACTH secretion, possibly by stimulating corticotropin releasing factor secretion from the hypothalamus. Cushing's disease seems to be due to defective hypothalamic regulation of ACTH release from the pituitary gland. cyproheptadine is a potent antagonist of serotonin and has been used successfully in some patients with Cushing's disease, although, generally, in women without radiological evidence of pituitary tumors. We report the successful use of cyproheptadine in a 54-year-old man with Cushing's disease due to pituitary basophil adenoma. Significant clinical and biochemical improvement was noted 45 days after treatment began. The results in this patient support our findings that cyproheptadine can be effective in patients with Cushing's disease due to pituitary tumors, as well as in preparing very ill patients for surgery or managing such patients until radiotherapy takes effect.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/10. A case of ruptured dissecting aneurysm 5 years after pituitary microsurgical treatment of Cushing's disease: autopsy findings in the hypothalamic-pituitary-adrenal axis.The patient was a 26-year-old man with Cushing's disease who underwent transsphenoidal microscopic surgery for a pituitary microadenoma. His postoperative course was uneventful, but he died suddenly five years after the operation. At autopsy, a ruptured dissecting aneurysm with marked atherosclerosis was observed in the aorta. In the pituitary, a small focus of adrenocorticotropic hormone (ACTH) producing adenoma, possibly residual adenoma, was detected and Crooke's degeneration was observed in the non-tumorous pituitary gland. But immunohistochemical patterns of pituitary hormones in the non-tumorous pituitary gland were normal and the adrenal cortex was unremarkable. In the hypothalamus, corticotropin-releasing hormone immunoreactivity was not detected and arginine vasopressin was sporadically positive. Considering these findings, this patient may have developed subclinical hypercortisolism due to the residual adenoma at the time of autopsy, despite clinical remission. Cushing's syndrome is considered to be a risk factor dissecting aneurysm, and in this case the metabolic changes in Cushing's disease may have influenced the development of the dissecting aneurysm. Periodic cardiovascular re-evaluations should therefore be performed when there is clinical remission of Cushing's syndrome.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
10/10. Coexistence of primary empty sella and silent corticotrophic adenoma.Primary empty sellae are rarely associated with an intact pituitary adenoma. Most of such cases are documented clinically and radiographically. This paper reports a case of coexistence of primary empty sella and a silent corticotrophic adenoma in a 61-year-old woman. The en bloc preparation of the sella turcica demonstrates the anatomical relationship of the empty sella and the flattened pituitary gland and the adenoma. The immunostain shows the presence of all five cell types of the adenohypophysis.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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