1/124. Competitive polymerase chain reaction for the determination of N-myc amplification in neuroblastoma: report of clinical cases.If an unfavorable prognosis is suspected in neuroblastoma, decision on a treatment protocol should be based on the N-myc copy number (12). We already demonstrated that the newly developed competitive polymerase chain reaction (competitive PCR) is a promising method for the determination of the N-myc copy number (6), and have started to use this competitive PCR procedure in neuroblastoma patients, together with fine-needle biopsy in selected cases. Seven children were studied. In one infant of 5 months of age whose tumor was diagnosed before undergoing mass screening for neuroblastoma, the competitive PCR procedure was performed with a fine-needle biopsy, and after obtaining a negative report on N-myc amplification within 48 hours, a regular protocol of treatment could be started without delay. We report that competitive PCR is a rapid and accurate method for the determination of the N-myc copy number, requiring only a small amount of material, and anticipate that competitive PCR will become the procedure of choice for the determination of N-myc copy number in neuroblastoma.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
2/124. 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 = 432.26640803087keywords = rhythm (Clic here for more details about this article) |
3/124. Adrenal adenocarcinoma and empty sella syndrome in a 37-year-old woman.The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. The patient underwent hormonal evaluation including circadian rhythm of cortisol, gonadotropin-releasing hormone/thyroid-stimulating hormone (GnRH/TRH) test, corticotropic-releasing hormone (CRH) test and dexamethasone suppression test. She also underwent pelvic and adrenal ultrasound examination, adrenal computed axial tomography (CAT) scan and cranial nuclear magnetic resonance (NMR). A mass about 10 cm in size was detected in the left adrenal region. The sella was empty and the pituitary displaced downward. Suspected adrenal adenocarcinoma was confirmed by histological examination after surgical removal of the mass. This case is of interest for physicians because of the mixed androgen and cortisol secretion of the adenocarcinoma in a hyperprolactinemic patient with empty sella. Moreover, it suggests the need to investigate the adrenal gland in patients with hyperprolactinemia and hirsutism.- - - - - - - - - - ranking = 216.13320401543keywords = rhythm (Clic here for more details about this article) |
4/124. Acute watery diarrhea as the initial presenting feature of a pheochromocytoma in an 84-year-old female patient.We report the case of an 84-year-old woman who was initially admitted to the emergency room of our institution for frank dehydration caused by acute and severe secretory diarrheas along with acidosis and hypokalemia. After extensive gastrointestinal investigations, the etiology of the diarrhea remained unclear. Because clinical symptoms and ionogram parameters worsened, despite intravenous fluids and electrolyte replacement, an abdominal CT scan was performed and unexpectedly revealed a 4.5-cm mass in the right adrenal gland. Several separate 24-hour urine catecholamines were shown to be highly elevated. The diagnosis of pheochromocytoma was confirmed by MIBG scintigraphy and MRI. Before the admission, the patient never experienced symptoms suggestive of pheochromocytoma, except dry mouth and fear of impending death on several occasions. After 2 weeks, the diarrhea stopped abruptly and spontaneously without specific medication but after adequate rehydration. The patient subsequently underwent surgical removal of the adrenal medullary mass. Postoperatively, urinary catecholamines returned to normal values. Immunohistochemical study of the tumor confirmed the diagnosis of pheochromocytoma and revealed the presence of VIP-positive cells organized as islets in scattered areas of the tissue. This case illustrates the protean mode of presentation of pheochromocytoma, as well as the ability of medullary neural crest-derived cells to produce various neuropeptides potentially responsible for a large variety of symptoms.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
5/124. 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 = 904.41431313474keywords = diurnal, rhythm (Clic here for more details about this article) |
6/124. pheochromocytoma in an incidentally discovered asymptomatic cystic adrenal mass.Cystic adrenal lesions can be either cortical or medullary, and distinguishing between these 2 types of lesions may be important in patient management. Pheochromocytomas, which are adrenal medullary neoplasms, typically manifest with hypertension, headaches, palpitations, tachycardia, sweating, and anxiety symptoms; however, 10% to 17% of patients with pheochromocytomas are asymptomatic. We describe a 67-year-old woman with lifelong headaches and recent persistent cough in whom a left cystic adrenal mass was incidentally discovered by computed tomography of the chest. A moderate increase in normetanephrine and total metanephrine values in two 24-hour urine samples suggested a pheochromocytoma. Computed tomography with use of contrast medium revealed ring enhancement of the cyst wall, a finding consistent with an adrenal medullary tumor. This report demonstrates the importance of repeated 24-hour urine samples to determine the metanephrine values together with contrast-enhanced computed tomography in a patient with nonspecific symptoms.- - - - - - - - - - ranking = 2keywords = hour (Clic here for more details about this article) |
7/124. Traumatic hemorrhage of occult pheochromocytoma: a case report and review of the literature.pheochromocytoma usually presents with gradual onset and mild to moderate symptoms, but may present acutely with severe symptoms. Hemorrhage into pheochromocytoma is a rare cause of acute presentation that is often devastating to patients. We describe the case of a 34-year-old woman with hemorrhage into a previously undiscovered pheochromocytoma following a fall on a patch of ice. This is the first reported case of hemorrhagic pheochromocytoma associated with traumatic injury. Despite removal of the tumor within 18 hours of presentation, the patient suffered severe complications of massive catecholamine excess, including shock, cardiomyopathy, and adult respiratory distress syndrome. Animal studies have shown that early treatment with alpha blockers can prevent some, if not all of these complications. Proper management of hemorrhagic pheochromocytoma should include a high index of suspicion with early diagnosis and treatment with alpha blockers and surgical resection of the tumor when the patient is stable enough to tolerate the procedure.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
8/124. A 35-year-old man with cerebral hemorrhage and pheochromocytoma: the second brain-dead organ donor in japan.A 35-year-old man was brought into the emergency room of Keio University Hospital by ambulance because of a sudden onset of coma. His glasgow coma scale was 3 and his blood pressure 150/100 mmHg. CT scanning revealed a subcortical hemorrhage 8 cm in diameter. His respiration deteriorated rapidly, and an emergency craniotomy was performed for hematoma removal and cerebral decompression. Postoperatively the patient remained in a deep coma (GCS = 3) requiring respiratory support. The family presented an organ donor card previously signed by the patient, and brain death was confirmed in accordance with japan's transplant law. As a result of two tests conducted six hours apart brain death was confirmed on the 5th postoperative day. With the family's consent, the donor's heart, kidneys and skin were removed for organ transplantation to be performed in other institutions. An autopsy was performed after the removal of the organs and skin. An extensive subgaleal hemorrhage was found in the left cerebral hemisphere, and microscopic examination revealed extensive necrosis with karyolysis of neuronal cells, but no viable neuronal cells were found in the cerebrum. The brain stem was marked by edema, hemorrhage, infarction necrosis and neuronal cell loss. The cerebellum was swollen and congested and showed autolysis of the granular layer. These findings suggested brain death syndrome with respirator brain. Other autopsy findings included a huge pheochromocytoma in the right adrenal gland, bilateral bronchopneumonia, liver congestion and fatty metamorphosis with four cavernous hemangiomas, and mild chronic lymphocytic thyroiditis. This patient was the second brain-dead organ donor and the first brain-dead patient to undergo postmortem examination in japan.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
9/124. A case report of the use of magnesium sulphate during anaesthesia in a patient who had adrenalectomy for phaeochromocytoma.INTRODUCTION: patients with phaeochromocytoma have haemodynamic instability during adrenalectomy. CLINICAL PICTURE: A case showing major swings of blood pressure during tumour handling. TREATMENT: magnesium sulphate infusion alone failed to prevent severe hypertension. OUTCOME: The patient had to be given phentolamine and sodium nitroprusside to control the severe hypertension. CONCLUSION: The greatest value of magnesium sulphate is in controlling catecholamine release at induction and intubation, and in association with other agents in controlling arrhythmias and hypertension during tumour handling.- - - - - - - - - - ranking = 216.13320401543keywords = rhythm (Clic here for more details about this article) |
10/124. Unusually aggressive rectal carcinoid metastasizing to larynx, pancreas, adrenal glands, and brain.Rectal carcinoids are slow-growing tumors. They metastasize when their size is more than 2 cm. Common sites of metastasis are the liver, lungs, and bones. Metastases to thyroid, pancreas, kidneys, adrenal glands, pituitary glands, posterior fossa, and spleen are very rare. We present the case of a 79-year-old white man with dysphagia and left vocal cord paralysis from a rapidly growing mass in his neck. Needle biopsy suggested thyroid anaplastic carcinoma, and the patient underwent total laryngectomy, total thyroidectomy, and left radical neck dissection. pathology showed undifferentiated carcinoid of the larynx. biopsy of a rectal mass suggested poorly differentiated carcinoma. Postoperatively the patient developed cardiac arrhythmias and died after 5 weeks. autopsy showed a 5-cm carcinoid of the rectum with extensive vascular invasion extending into the perirectal fat. There was metastatic disease to both lungs, liver, pancreas, both adrenal glands, peritoneum, subcutaneous tissues of thorax and abdomen, ribs, vertebrae, skull, and the leptomeninges of the cerebrum. Rectal carcinoids may present a variable histologic picture. Poorly differentiated tumors can present with widespread metastases and have poor prognosis. Extensive surgery may not improve the survival of patients with this pattern of unusually aggressive carcinoid.- - - - - - - - - - ranking = 216.13320401543keywords = rhythm (Clic here for more details about this article) |
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