1/138. A case of aldosterone-producing adenoma with severe postoperative hyperkalemia.It is known that some patients with primary aldosteronism show postoperative hyperkalemia, which is due to inability of the adrenal gland to secrete sufficient amounts of aldosterone. However, hyperkalemia is generally neither severe nor prolonged, in which replacement therapy with mineralocorticoid is seldom necessary. We report a case of a 46-year-old woman with an aldosterone-producing adenoma associated with severe postoperative hyperkalemia. After unilateral adrenalectomy, the patient showed episodes of severe hyperkalemia for four months, which required not only cation-exchange resin, but also mineralocorticoid replacement. plasma aldosterone concentration (PAC) was low, although PAC was increased after rapid ACTH test. Histological examination indicated the presence of adrenocortical tumor and paradoxical hyperplasia of zona glomerulosa in the adjacent adrenal. immunohistochemistry demonstrated that the enzymes involved in aldosterone synthesis, such as cholesterol side chain cleavage (P-450scc), 3beta-hydroxysteroid dehydrogenase (3beta-HSD), and 21-hydroxylase (P-450c21), or the enzyme involved in glucocorticoid synthesis, 11beta-hydroxylase (P-450c11beta), were expressed in the tumor, but they were completely absent in zona glomerulosa of the adjacent adrenal. These findings were consistent with the patterns of primary aldosteronism. serum potassium level was gradually decreased with concomitant increase in PAC. These results suggest that severe postoperative hyperkalemia of the present case was attributable to severe suppression of aldosterone synthesis in the adjacent and contralateral adrenal, which resulted in slow recovery of aldosterone secretion. It is plausible that aldosterone synthesis of adjacent and contralateral adrenal glands is severely impaired in some cases with primary aldosteronism, as glucocorticoid synthesis in cushing syndrome.- - - - - - - - - - ranking = 1keywords = adrenocortical (Clic here for more details about this article) |
2/138. Recurrent adrenocortical carcinoma in a child.An 8-month-old girl presented with clitoromegaly, cushingoid features and a large abdominal tumour. ultrasonography (US) and computed tomography (CT) of the abdomen revealed a tumour of the left suprarenal gland, 12x11x7 cm in size. serum levels of cortisol, testosterone and DHEA-S, and urinary extretion of 17-ketosteroids and 17-hydroxycorticoids were increased. Complete removal of the tumour was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically. Three months after the first operation, a recurrent tumour of the left renal hilus, 23x15 mm in size, was identified by US and verified by aspiration biopsy. The tumour was removed by the transabdominal route. In this report, we discuss the diagnosis and the treatment of this rare disease.- - - - - - - - - - ranking = 5keywords = adrenocortical (Clic here for more details about this article) |
3/138. Subclinical cushing syndrome due to adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia: changes in plasma cortisol levels during long-term follow-up.A 63-year-old man with bilateral adrenal tumors was treated for hypertension and diabetes mellitus. Endocrinologic examination during follow-up revealed the production of cortisol to be independent of the regulation of the hypothalamo-pituitary-adrenal axis. Therefore, he was diagnosed as having subclinical cushing syndrome, and these tumors were removed. Adrenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) was endocrinologically and histopathologically diagnosed. This is the first report demonstrating that endocrinologic changes of AIMAH can be observed during long-term follow-up.- - - - - - - - - - ranking = 4keywords = adrenocortical (Clic here for more details about this article) |
4/138. Laparoscopic partial adrenalectomy in patients with hereditary forms of pheochromocytoma.PURPOSE: patients with von hippel-lindau disease are predisposed to multiple bilateral adrenal pheochromocytoma. In these patients partial adrenalectomy may preserve adrenocortical function and avoid the morbidity associated with medical adrenal replacement. We report our experience with such cases. MATERIALS AND methods: Laparoscopic partial adrenalectomy was performed in patients with von hippel-lindau disease and pheochromocytoma when there was evidence of normal adrenocortical tissue on preoperative imaging or intraoperative examination. Suture ligature or a harmonic scalpel was used to excise the tumors, leaving a 2 to 3 mm. margin of normal tissue. RESULTS: Two patients underwent laparoscopic partial adrenalectomy and 1 laparoscopic bilateral partial adrenalectomy with preservation of normal adrenocortical tissue. Seven pheochromocytomas were removed. Laparoscopic ultrasound was essential for localizing 2 pheochromocytomas that were not visualized by the camera. Median operative time was 324 minutes, blood loss 100 cc and parenteral narcotic requirement 22 mg. morphine equivalents. No patient required hydrocortisone replacement. There has been no pheochromocytoma recurrence during short-term followup. CONCLUSIONS: Laparoscopic partial adrenalectomy is technically feasible in patients with a hereditary form of pheochromocytoma, and may preserve adrenocortical function. Laparoscopic ultrasound was necessary to identify 2 of the 7 pheochromocytomas removed.- - - - - - - - - - ranking = 4keywords = adrenocortical (Clic here for more details about this article) |
5/138. Adrenal myelolipoma.A myelolipoma of the adrenal gland is reported. Diagnostic features include (1) hyperlucent mass on intravenous pyelography with renal displacement, (2) an avascular mass on arteriography and venography, (3) solid tissue mass on B-mode ultrasonography, and (4) no clinical or chemical evidence of adrenal hyperfunction.- - - - - - - - - - ranking = 0.3383615200981keywords = hyperfunction (Clic here for more details about this article) |
6/138. Adrenal tumor presenting as precocious puberty.We present a case report of a two and a half-year-old boy who presented with precocious puberty. A clinical diagnosis of congenital adrenal hyperplasia was made. Patient was investigated and found to have an adrenocortical tumor. The tumor was about 7 cms in diameter. The tumor was secreting androgens, 17OHP and cortisol. This is an unusual array of hormones to be secreted by an adrenal tumor.- - - - - - - - - - ranking = 1keywords = adrenocortical (Clic here for more details about this article) |
7/138. Sustained remission of metastatic adrenal carcinoma during long-term administration of low-dose mitotane.We present our experience with two female patients suffering from metastatic, recurrent adrenocortical carcinomas, to whom o,p'-DDD (mitotane) was administered for unusually long duration. The first patient received mitotane as monotherapy after relapse (in doses ranging from 3 to 6 g/day initially and 1 g/day thereafter, for 13 yr). The second patient presented with metastatic disease and underwent radical surgical excision of the adrenal. mitotane was administered initially at 2.5 g/day, and the dose was gradually lowered over 8 yr to 1 g/day, without interruption. Both patients tolerated the medication well, regardless of the daily dosage, with complaints limited to epigastric pain and nausea, while their disease has been kept under control for 14 and 16 yr, respectively. The blockade of steroid synthesis with mitotane resulted in hypercholesterolemia in both patients and in premature menopause in the second patient; however, these abnormalities were taken care of with the appropriate therapy. The excellent follow-up of these patients suggests that even in hopeless cases with metastatic adrenocortical carcinoma, mitotane should be administered for very long periods of time as it can be well-tolerated and may be beneficial in the long run.- - - - - - - - - - ranking = 2keywords = adrenocortical (Clic here for more details about this article) |
8/138. testosterone-secreting gonadotropin-responsive adrenal adenoma and its treatment with the antiandrogen flutamide.A 55-year-old woman with virilization had an appreciably elevated testosterone level, which was not suppressed by dexamethasone, but was increased by stimulation with human chorionic gonadotropin (hCG). ultrasonography and computed tomography revealed an adenoma 2.5-3.0 cm in diameter in the right adrenal gland. The patient was treated with the antiandrogen flutamide in a daily dose of 500 mg for 4 months. A substantial regression of her hirsutism was observed during flutamide administration, but the serum testosterone level remained high. Right adrenalectomy was performed. Histologically, the tumor proved to be an adrenocortical adenoma of zona reticularis type. The adenoma tissue contained specific hCG receptors (187 fmol/g). The steroid concentration in the tumor tissue was examined by means of high pressure liquid chromatography-radioimmunoassay (HPLC-RIA). A significantly increased testosterone content was detected, and the levels of its precursors, androstenedione and dehydroepiandrosterone, were also elevated. Following adrenalectomy, serum testosterone concentration decreased to the normal level. The mechanism of the inappropriate regulation in the testosterone production of the adrenal tumor has not been fully elucidated.- - - - - - - - - - ranking = 1keywords = adrenocortical (Clic here for more details about this article) |
9/138. Evaluation and surgical resection of adrenal masses in patients with a history of extra-adrenal malignancy.BACKGROUND: Adrenal abnormalities are often identified on imaging studies performed during the staging of patients presenting with a new malignancy or restaging of patients with a history of a malignancy. methods: We reviewed the records of patients who underwent surgical resection of an adrenal mass identified in the setting of previously or newly diagnosed extra-adrenal malignancy. RESULTS: Eighty-one patients with an adrenal mass and recently diagnosed malignancy (n = 24) or history of a malignancy (n = 57) underwent adrenalectomy. In 42 patients (52%) the adrenal mass was a metastasis. In 39 patients (48%) the adrenal mass was an additional primary adrenal tumor process: 19 pheochromocytomas, (14 syndrome-associated, 5 sporadic), 13 cortical adenomas, 3 adrenocortical carcinomas, 2 ganglioneuromas, and 2 cases of nodular hyperplasia. CONCLUSIONS: In this series nearly half of the patients with cancer and an adrenal mass had adrenal pathologic condition independent of their primary malignancy. Despite the presence of a newly diagnosed malignancy or history of malignancy, all patients with an adrenal mass should undergo a standard hormone evaluation to confirm that the mass is not a functional neoplasm. An assumption that the adrenal mass is metastatic disease will be wrong in up to 50% of such patients.- - - - - - - - - - ranking = 1keywords = adrenocortical (Clic here for more details about this article) |
10/138. Development of overt Cushing's syndrome in patients with adrenal incidentaloma.OBJECTIVE: The natural course of adrenal incidentalomas, especially those with subclinical autonomous glucocorticoid production, i.e. subclinical Cushing's syndrome, and the risk that such conditions will evolve towards overt Cushing's syndrome are unknown. DESIGN: Longitudinal follow-up evaluation of a series of 284 consecutive patients with adrenal incidentaloma. methods AND RESULTS: Out of 284 consecutive patients with adrenal incidentaloma studied at our Institution in the last 15 years, 98 patients (23 with subclinical hypercortisolism) underwent surgery. Of 130 non-operated patients with a follow-up of at least 1 year, eight had subclinical hypercortisolism at diagnosis. We describe in detail four patients who developed overt Cushing's syndrome after 1-3 years of follow-up. Only one of these patients had subclinical hypercortisolism at first diagnosis. Estimated cumulative risk for a non-secreting adrenal incidentaloma to develop subclinical hyperfunction was 3.8% after 1 year and 6.6% after 5 years. For patients with masses with subclinical autonomous glucocorticoid overproduction, estimated cumulative risk to develop overt Cushing's syndrome was 12.5% after 1 year. CONCLUSIONS: In patients with adrenal incidentalomas the risk of progression towards overt Cushing's syndrome is not low, at variance with previous reports. A careful biochemical and hormonal follow-up is advisable in all patients who do not need surgery at first presentation.- - - - - - - - - - ranking = 0.3383615200981keywords = hyperfunction (Clic here for more details about this article) |
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