1/22. GIP-dependent adrenal Cushing's syndrome with incomplete suppression of ACTH.ACTH-independent Cushing's syndrome may be due to the development of ectopic hormone receptors in adrenal tissue. Thus, in food-dependent Cushing's syndrome the adrenals aberrantly express receptors for gastric inhibitory polypeptide (GIP). We present the case of a 60-year-old woman with food-dependent Cushing's syndrome whose cortisol levels increased after stimulation with CRH. In this patient with Cushing's syndrome the finding of low basal plasma cortisol levels in the late night and early morning as well as a paradoxical rise of plasma cortisol during a 7-h infusion with dexamethasone (carried out without any restriction in food intake), suggested that cortisol production was stimulated at times of food intake. Hourly measurements of plasma cortisol for 48 h revealed prominent meal-related peaks. A plasma cortisol response, elicited by oral glucose administration, could be prevented by octreotide. plasma ACTH was low or undetectable. CRH administration was followed by a ACTH response from 3 to 16 ng/l and a plasma cortisol response from 230 to 680 nmol/l. octreotide treatment for nearly five months induced a partial clinical and biochemical remission. Total bilateral adrenalectomy was performed. The left adrenal was grossly enlarged (7 x 5.5 x 4 cm) and the right adrenal was slightly enlarged (6 x 4 x 1.8 cm). microscopy revealed bilateral nodular hyperplasia. Cell suspensions of adrenal tissue from the patient did respond in a dose-dependent fashion to stimulation with GIP and were very sensitive to stimulation with synthetic ACTH1-24. However, CRH had no significant effect on cortisol production in vitro. Using RT-PCR amplification and cDNA hybridization, GIP receptor was found to be overexpressed in the left and right adrenal tissues from this patient as compared to adrenal tissues from a normal individual or from non GIP-dependent adrenal Cushing's syndrome. There was no evidence of presence of adrenal CRH receptors. Thus, in this patient with food-dependent Cushing's syndrome, the CRH-induced plasma ACTH and cortisol response is probably mediated by an incomplete suppression of the HPA axis as a result of the intermittent food-dependent nature of Cushing's syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/22. Pitfall of the accessory spleen.Two patients, one with insulinoma and one with Cushing's syndrome, are presented. Biochemical evaluation readily suggested the correct diagnosis. During radiologic imaging, the anatomic abnormality giving rise to these diseases, i.e. a pancreatic islet cell tumor, and an adrenal adenoma, at first were mistakenly interpreted as an accessory spleen on the basis of specific computed tomography and magnetic resonance imaging appearances. The insulinoma was identified as such during laparotomy, whereas additional jodo-cholesterol scintigraphy revealed the real nature of the lesion in the patient with Cushing's syndrome. Both patients were operated successfully.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/22. Management approaches to adrenal incidentalomas (adrenalomas). A view from Athens, greece.We believe the management of adrenalomas should include the following: 1. A detailed history and physical examination to detect subtle evidence of hormonal hypersecretion or the possibility of metastatic carcinoma 2. Hormonal studies, such as The short dexamethasone suppression test (2 mg of dexamethasone) followed by a high-dose dexamethasone suppression test (8 mg), CRH assay, and analysis of the diurnal cortisol rhythm if serum cortisol is greater than 3 micrograms/dL 24-Hour (or spot) urinary catecholamine metabolites (metanephrine and normetanephrine) plasma aldosterone level and renin activity in the hypertensive or normotensive patient with serum potassium less than 3.9 nmol/L 3. Additional imaging studies, such as MR imaging 131I-iodocholesterol (NP59) scanning to detect a subclinically functioning adenoma or carcinoma (MIBG scanning is rarely indicated) The role of FNA is limited. This modality may be helpful only in the patient with coexistent extradrenal carcinoma to confirm adrenal metastasis. Although genetic and molecular biology studies do not have wide clinical application, they should be encouraged and supported. Once all of these data are collected, the clinician should perform the following two steps: 1. All clearly nonfunctioning adrenalomas that are not suspicious for malignancy should be observed for several years, mainly with hormonal studies, until their secretory and benign nature is confirmed. 2. All patients with adrenalomas with evidence of subclinical function or potential for malignancy (using size, imaging, FNA, and molecular biology criteria) should undergo laparoscopic adrenalectomy for definitive diagnosis and therapy. The age, the overall medical condition, and the anxiety of the patient should be considered in the decision to operate on a patient with an adrenaloma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/22. Three patients with adrenal tumors having been treated simply for diabetes mellitus.Three patients with functional adrenal tumors, Cushing's syndrome, primary aldosteronism and pheochromocytoma, who underwent adrenalectomy and were subsequently cured, were studied. All these patients had been treated for diabetes for several years before the diagnosis of adrenal tumors. In each case the state of diabetes before and after surgery, including parameters of insulin secretion and insulin resistance, was compared to demonstrate how the adrenal disorder influenced the nature of diabetes. In the case of Cushing's syndrome the hypercortisolemia caused insulin resistance in the peripheral tissues. In the case of primary aldosteronism, excessive production of aldosterone diminished insulin secretion possibly through hypokalemia. pheochromocytoma affected both insulin secretion and insulin sensitivity through hypersecretion of catecholamines. In all these patients the adrenal tumors were found in clinical contexts other than management of diabetes itself. By careful retrospective review of these three patients' history, several important points that might have drawn the physician's attention to the underlying adrenal disorders were pointed out. These included past history of acute myocardial infarction with onset at unexpectedly young age in the case of Cushing's syndrome and unexpectedly high insulin resistance for the patient's body mass index in the case of pheochromocytoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/22. Simultaneous bilateral laparoscopic adrenalectomy in ACTH-independent macronodular adrenal hyperplasia.Laparoscopic surgery for urological conditions has now become popular worldwide. The case is reported of a 56-year-old woman who underwent simultaneous bilateral laparoscopic adrenalectomy for adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia (AIMAH), followed by autotransplantation of resected adrenal gland fragments. Simultaneous laparoscopic adrenalectomies seem feasible for a patient with AIMAH because of its minimally invasive nature. However, autotransplantation of adrenal fragments failed in this patient with AIMAH.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/22. Corticotroph pituitary carcinoma: case report and literature review.Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-year-old female first presented with an invasive and clinically non-functioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing's disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke's hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/22. Androgen-secreting adrenal adenomas.BACKGROUND: The androgen source in women with hirsutism and signs of virilism may be the ovary or adrenal gland. CASES: Three patients with androgen excess are reported. Two had hyperandrogenemia and cushing syndrome with an adrenal mass greater than 5.5 cm; the third had a small adrenal adenoma secreting only testosterone and responsive to human chorionic gonadotropin. In all cases, the pathologic report from surgery and the long-term resolution of symptoms confirmed the benign nature of the tumors. CONCLUSION: Basal and dynamic hormonal tests cannot precisely differentiate ovarian from adrenal tumors. Adrenal adenomas must be considered as a cause of hyperandrogenic syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/22. Adrenal black adenoma associated with Cushing's syndrome.We report a case of adrenal black adenoma associated with Cushing's syndrome. A 41-yr-old man presented to our hospital with a 6-yr history of severe hypertension and general fatigue, and a 1-mo history of diabetes mellitus. physical examination disclosed cushing-oid manifestations. His serum cortisol concentrations ranged from 14.0 to 15.4 microg/dL, with an ACTH level <5 pg/mL. Urinary free cortisol level was increased (125 microg/d). Cortisol was not suppressed on the overnight 1 mg oral dexamethasone suppression test (DST), 2-d low-dose DST, and 2-d high-dose DST. Abdominal computed tomography and magnetic resonance imaging studies revealed a solid round tumor approx 3 cm in diameter, located in the left adrenal gland. Left adrenalectomy was performed; the surgical specimen revealed a black ade-noma consisting of compact cells within numerous pigments that seemed to be lipofuscin in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/22. Prognostic indicators in an aggressive pituitary Crooke's cell adenoma.OBJECTIVE: To investigate prognostic indicators in an aggressive Crooke's cell adenoma of the pituitary. methods: The surgically removed tumor was studied by histology, immunohistochemistry and transmission electron microscopy. RESULTS: An aggressive invasive sellar tumor removed by repeated surgeries from a 43-year-old woman with pituitary related Cushing's disease was classified as a Crooke's cell adenoma of the pituitary. The application of several cell proliferation markers confirmed the aggressive nature of the tumor. CONCLUSIONS: The investigation of the present case provides additional evidence that pituitary Crooke's cell adenomas may possess aggressive behavior.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/22. In vivo and in vitro studies in a patient with cyclical Cushing's disease showing some responsiveness to bromocriptine.In vivo and in vitro studies were carried out in a 37-year old female with cyclical Cushing's disease. Preoperative studies revealed periodic secretions of urinary corticosteroids occurring with a cyclicity of 2-3 weeks. On transsphenoidal surgery, a microadenoma was visualized in the anteroinferior portion of the anterior pituitary. Gel filtration analyses of the adenoma and surrounding tissues revealed increased concentrations of beta-endorphin and an activated conversion of beta-lipotropin to beta-endorphin in the adenoma compared with the surrounding tissues. These findings were in agreement with the characteristics previously reported for corticotroph adenomas. However, unexpectedly, concentrations of ACTH and beta-lipotropin in the adenoma were only slightly higher than those in the surrounding tissues. Precise mechanisms underlying this unusual finding were elusive, but it may have been due to the periodic nature of her hypercortisolism. In addition, this patient was reproducibly responsive to bromocriptine (2.5 mg, per os) with a reduction of the plasma cortisol level. Although this may suggest an intermediate lobe subtype of Cushing's disease as proposed by Lamberts' group, our case did not have any other characteristic suggestive of this proposed variant. However, it is tempting to speculate that cyclical changes in the central dopaminergic tone may have been at least a partial trigger for the periodic hormonogenesis in this patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
| | Next -> |