1/238. The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis.A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/238. Posttraumatic hypertension secondary to adrenal hemorrhage mimicking pheochromocytoma: case report.We report the case of a 68-year-old man who presented with a mass 3 x 4 cm in size located in the right adrenal gland together with extreme hypertension, tripled urine levels for normetanephrine, and normal plasmatic levels of metanephrines. The patient had suffered a fall from a height of 2.5 meters before hospitalization. [123I]MIBG-scan was repeatedly positive in the area of the right adrenal gland. At laparotomy under alpha-adrenergic blocking agents, the suspected pheochromocytoma was histologically confirmed as hematoma. After resection of the adrenal gland, blood pressure returned to normal without drug therapy as did metanephrine levels in urine. Although adrenal insufficiency after distension of the gland caused by hemorrhage has been reported, there are no data available regarding the mimicking of a hormonally active pheochromocytoma. We conclude that intra-adrenal pressure rise caused by hematoma may cause partial ischemic necrosis to the gland but may also induce reactive hyperplasia with periodic excessive secretion of catecholamines. This interpretation is consistent with the finding that plasma levels of catecholamines were normal in contrast to the urinary normetanephrines in the presented case. It might be worthwhile to investigate patients with intra-adrenal hemorrhage immediately after sustaining multiple injuries and in the posttraumatic course of several months up to 1 or more years together with verification of abnormal urinary excretion of metanephrines as a sign of impaired adrenal function.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
3/238. Malignant pheochromocytoma with multiple hepatic metastases treated by chemotherapy and transcatheter arterial embolization.A 62-year-old Japanese male developed multiple hepatic metastases two years after resection of pheochromocytoma of the right adrenal gland. Transcatheter arterial embolization (TAE) was performed for the purpose of the treatment of hepatic metastases resistant to 27 cycles of combined chemotherapy consisting of cyclophosphamide, vincristine, and dacarbazine. After TAE, the hepatic metastatic lesions decreased in size and hypertension passed its crisis. The present case suggests the utility of TAE for multiple hepatic metastases under careful blood pressure monitoring.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/238. hypertension and virilization caused by a unique desoxycorticosterone- and androgen-secreting adrenal adenoma.We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. dexamethasone (DEX) was administered, therefore, which partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a right adrenal tumor. Post surgery, the patient promptly resumed menses and became normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of delta 4 and delta 5 steroids, present here, may favor a tumor. This case also allows speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/238. Unsuspected pheochromocytoma with abdominal aortic aneurysm--a case report.This case report describes the peri-operative management of a 65-year-old man with an unsuspected pheochromocytoma. He underwent emergent surgery for a rupturing abdominal aortic aneurysm. During surgery his blood pressure changed dramatically and was resistant to drug treatment. A pheochromocytoma was suspected, but the emergency precluded immediate investigation. A second hypertension episode occurred in the intensive care unit, and CT revealed an abnormal adrenal mass. Surgery for the pheochromocytoma was carried out successfully later, with the hypertension being managed very carefully. We considered that exposure to extraordinary catecholamine levels from the pheochromocytoma might have contributed to the development of the abdominal aortic aneurysm.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/238. Anesthetic management for pheochromocytoma resection using spinal cord stimulation and intravenous nicardipine--a case report.We have used spinal cord stimulation (SCS), diazepam and nitrous oxide for maintenance of general anesthesia. blood pressure was maintained by bolus administration of nicardipine for the removal of pheochromocytoma. Both SCS and nicardipine reduced systemic vascular resistance and SCS increased cardiac output. However, neither SCS nor nicardipine could inhibit the release of norepinephrine. SCS proved to be useful as one of anesthetic technique during the removal of pheochromocytoma, and also in the management of postoperative pain and the prevention of complications.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/238. aldosterone-producing adenoma without hypertension: a report of two cases.Normotensive primary hyperaldosteronism is exceedingly rare. We report two new cases of this syndrome in two middle-aged women, one of Asian origin. The presenting signs were tetany in one case and an adrenal mass in the other. Neither patient had hypertension, despite repeated measurements with a manual armlet. A typical biological profile of primary hyperaldosteronism was demonstrated in both patients, including hypokalemia with inappropriate kaliuresis, elevated resting plasma aldosterone, and undetectable plasma renin activity. The circadian rhythm of blood pressure was studied by ambulatory monitoring pre- and post-operatively. It confirmed the lack of hypertension, but the circadian rhythm of blood pressure was lost before surgery in one patient. Surgical removal of the histologically typical aldosterone-producing adenomas normalized the kalemia. The main finding in these two patients was spontaneously low blood pressure in the post-operative period. This suggests that excess aldosterone induced relative hypertension in these patients whose blood pressure was spontaneously very low. Genetic screening for dexamethasone-sensitive hyperaldosteronism was negative in both patients.- - - - - - - - - - ranking = 4keywords = pressure (Clic here for more details about this article) |
8/238. Behcet's disease and pheochromocytoma.A 46-year-old black female with a history of Behcet's disease was admitted with a diagnosis of abdominal pain. She had complained of palpitations, diaphoresis, anxiety, and fatigue for years which had been variously attributed to the perimenopause and her Behcet's. During her hospital evaluation, she had an episode of supraventricular tachycardia associated with severely elevated blood pressure. Subsequent evaluation revealed a large pararenal mass which was found to be a pheochromocytoma. Search of six databases revealed this to be only the second case report in the English literature of a pheochromocytoma in association with Behcet's disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/238. diagnosis and management of pheochromocytoma during pregnancy: a case report.pheochromocytoma is known to increase morbidity and mortality. We describe a case of pheochromocytoma during pregnancy. A patient was transferred to our hospital during gestational week 15 with severe hypertension, acute pulmonary edema, and cardiomyopathy. One day after transfer, she had a spontaneous abortion of the fetus. One week after hospital transfer, she developed acute dyspnea, supraventricular tachycardia degenerating into ventricular tachycardia, and respiratory failure requiring mechanical ventilation. pheochromocytoma caused by a right adrenal mass was diagnosed. The patient was treated with titrated doses of phenoxybenzamine, intravenous nicardipine, and metyrosine over a period of 3 weeks with resultant stabilization of her blood pressure. She underwent a successful right adrenalectomy 1 month after her initial presentation. Four months after surgery, all antihypertensive medications were discontinued and her blood pressure remained stable 1 year after the surgery. This case describes the maternal morbidity and fetal mortality that may be associated with pheochromocytoma during pregnancy.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
10/238. Noncardiogenic pulmonary edema as the chief manifestation of a pheochromocytoma: a case report of MEN 2A with pedigree analysis of the RET proto-oncogene.Pheochromocytomas are rare neoplasias of the adrenal medulla which generally present with paroxysmal or sustained hypertension. Cardiogenic pulmonary edema is a common feature of these tumors, but few cases have been described with noncardiogenic pulmonary edema. We report a pheochromocytoma with the principle manifestation of noncardiogenic pulmonary edema and characterize a genetic lesion associated with the disorder. A 30-year-old man was admitted with abdominal pain and breathlessness. x-Ray examination of the chest revealed a massive, diffuse infiltration of the left lung without cardiomegaly. No paroxysmal blood pressure fluctuations or heart failure were evident during the entire course, and the infiltrate and dyspnea resolved in three days without inotropic or diuretic agents. serum norepinephrine and epinephrine levels were elevated twenty and fifty times above normal, respectively. The patient was ultimately diagnosed with multiple endocrine neoplasia type 2a (MEN 2A). Mutations in the RET proto-oncogene have been described recently in patients with MEN 2A. mutation analysis of selected RET exonic sequences identified a germline mutation at codon 634 in exon 11 of the RET proto-oncogene. The mutation introduces a transition encoding a non-conservative substitution from TGC (Cys) to CGC (Arg) and creates a novel restriction site recognized by HhaI. We further screened for this mutation among four of the proband's relatives by HhaI restriction analysis. One asymptomatic family member was identified who subsequently elected prophylactic total thyroid removal. Histological examination of this specimen confirmed the presence of medullary thyroid carcinoma.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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