Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 14-year-old girl presented with malignant hypertension. Physical examination and abdominal computerized tomography revealed a paraaortic mass. Urinary catecholamines, serum renin, and aldesterone levels were high. The mass was totally excised and a paraganglioma of the organ of Zuckerkandl was confirmed. The hypertension resolved after surgery.
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PMID:Malignant hypertension and paraganglioma in a 14-year-old girl. 993 76

Paragangliomas are infrequent, usually benign tumors developed from neuroectoderm cells. The neck is the most common location, although some cases arise within the abdominal cavity, usually in the retroperitoneal space. We report five cases with bone metastases. In three patients, convincing evidence was obtained that the primary was in the retroperitoneal space. Clinical manifestations of metastatic bone disease occurred up to 17 years after the diagnosis of paraganglioma. Useful data were obtained from plain radiographs, magnetic resonance imaging, serum and urine catecholamine assays, and above all meta 123iodobenzylguanidine scintigraphy. Histologic and immunohistochemical studies of the lesion yielded the definite diagnosis. Surgery and radiation therapy are the two mainstays of therapy. Although rare, metastatic forms of paraganglioma should be borne in mind. This diagnosis should be entertained in patients with bone lesions and recent-onset arterial hypertension, irrespective of whether they report a history of surgery for a tumor, and even if this tumor was removed many years earlier and labeled benign.
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PMID:Bone metastases from a paraganglioma. A review of five cases. 1008 67

A 45-year-old woman with a posterior mediastinal tumor underwent right thoracotomy for resection, developing hypertension and difficult-to-control tachycardia while the tumor was being manipulated. A catecholamine-secreting tumor was suspected, the operation halted, and the patient prepared for surgery at a later time. The tumor was a mediastinal paraganglioma and the final outcome was satisfactory. Risk related to anesthesia is high in such patients, with perioperative mortality ranging from 40 to 85%. Correct diagnosis and appropriate preoperative drug preparation with adrenergic receptor blockers appreciably decreases morbidity and mortality related to surgery. We discuss the effect of labetalol on such tumors and describe our observations.
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PMID:[Intraoperative diagnosis of a mediastinal paraganglioma: Anesthetic management]. 1036 14

The authors describe the case of a 19-year-old female patient with an abdominal paraaortic extra-adrenal pheochromocytoma (paraganglioma), presenting arterial hypertension. The predominant catecholamine produced by the tumor was norepinephrine (4110 pg/ml; normal < 450 pg/ml). 131I metaiodobenzylguanidine (131I-MIBG), computed tomography and magnetic resonance imaging allowed location and characterization of the tumor. Histologically the tumor (weight = 34.2 g; 5.8 x 4 x 3 cm) was a typical pheochromocytoma.
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PMID:Extra-adrenal pheochromocytoma (paraganglioma). 1073 97

Paragangliomas are rare neoplasms of neural crest origin arising in the chromaffin (pheochromocytoma) and chemoreceptor (chemodectoma) tissues. Only a few cases of paragangliomas have been reported in the heart. Most of the cardiac paragangliomas are located in, or adjacent to, the left atrium. The biological activity indicative of catecholamine production has rarely been shown in cardiac paragangliomas. Patients with cardiac paragangliomas may present with hypertension, or with various obstructive or compressive symptoms, depending on the location of the tumour. A left atrial paraganglioma compressing the esophagus causing dysphagia is presented. A 37-year-old female patient underwent work-up for dysphagia to solids. Transesophageal echocardiography showed the presence of a large mass in the dilated left atrium. The tumour was removed completely intact and was a benign noncatecholamine-secreting paraganglioma. The patient's dysphagia was relieved. The clinical perspectives of cardiac paraganglioma and cardiac dysphagia are discussed.
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PMID:A left atrial paraganglioma patient presenting with compressive dysphagia. 1074 2

Pulsatile tinnitus (PT) is a perception of a rhythmical sound that is synchronous with the heartbeats. Despite being seen rarely in daily practice, frequently it is associated with identifiable causes, thus warranting special attention in regard to the etiological diagnosis. PT results from blood flow turbulence, which in turn results from changes in flow velocity or in the vessel lumen. One of the most important causes of PT is the paraganglioma, a vascular tumor that appears as a reddish retrotympanic mass. However, a normal tympanic membrane mandates differentiating among other diagnoses, such as arteriovenous malformations or fistulas, intracranial or extracranial aneurysms, a high or dehiscent jugular bulb, and persistent stapedial artery. Owing to the progress of radiological evaluation, magnetic resonance angiography (MRA) has proven to be excellent for evaluating vascular diseases. From January 1995 to June 1997, the authors prospectively studied 16 patients with PT and normal otoscopic examination. The study comprised 1 male and 15 female patients (ages 25-71 years; mean age, 42.5 years). All were subjected to MRA evaluation, which revealed the etiological diagnosis in 13 cases (81.25%), including 2 aneurysms and 1 case of intracranial hypertension. Of the 13 patients 9(69.23%) presented with at least one variation of vascular anatomy of the skull, showing a close correlation, in most cases, with the side on which PT occurred. Our results confirm that MRA is an excellent primary screening modality for patients with PT and normal otoscopic findings. The authors point out the importance of making etiological diagnoses in such cases, suggesting that variations of the vascular anatomy of the skull are a possible etiology.
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PMID:Magnetic Resonance Angiography in Pulsatile Tinnitus: The Role of Anatomical Variations. 1075 99

Paragangliomas are uncommon tumours that comprise less than 0.1% of all primary bladder neoplasms. Approximately half of the patients present the clinical triad of hypertension, haematuria and attacks associated with micturition. About 10% of vesical paragangliomas behave malignantly. This may be manifested as local invasion or metastatic spread, particularly to lymph nodes and lung. The best therapy remains total excision and partial cystectomy is considered adequate treatment for most cases. We report a case of paraganglioma of the bladder with extramural appearance in a 39-year-old man. Clinical symptoms were hypertension, dysuria and a well-defined hypogastric mass. One year after complete removal of the tumour, the patient is asymptomatic and normotensive, with normal catecholamine and vanillylmandelic acid (VMA) levels.
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PMID:Paraganglioma of the bladder: report of a case. 1080 76

Thoracic paragangliomas are a rare cause of hypertension. We report the occurrence of a sporadic benign norepinephrine-producing branchiomeric paraganglioma in a 32-year-old man with paroxysms of hypertension. After localization by iodine 123-metaiodobenzyl-guanidine scintigraphy and magnetic resonance imaging, the paraganglioma was resected successfully below the right pulmonary artery through a right-sided posterolateral thoracotomy. The particular location was consistent with a branchiomeric paraganglioma in an extremely rare extrapulmonary location.
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PMID:Resection of a branchiomeric paraganglioma at a rare extrapulmonary location. 1109 31

Paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up.
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PMID:Paraganglioma manifesting as shock: a case report. 1139 18

Paragangliomas are rare tumors that arise from extraadrenal chromaffin cells. We examined the clinical characteristics, location, treatment, and outcome of 236 patients (141 females, 60%) with 297 benign paragangliomas evaluated at the Mayo Clinic during 1978-1998. The mean age (+/-SD) at diagnosis was 47 +/- 16 yr. Of the 297 paragangliomas, 205 were in the head and neck region, and 92 were below the neck. Paragangliomas were discovered and diagnosed incidentally on imaging studies in 9% of patients. Biochemical screening was performed in 128 patients; 40 patients (17% of the total and 31% of those screened) had hyperfunctional tumors. Of the 40 patients with tumoral catecholamine excess, 38 had documented hypertension. In patients identified with catecholamine-secreting paragangliomas, the sensitivities achieved by measurements in the 24-h urine collection were 74% for total metanephrines, 84% for norepinephrine, 18% for dopamine, and 14% for epinephrine. Multiple imaging modalities were used for tumor localization. The false negative rates were 0% for magnetic resonance imaging, 5.8% for computed tomography, 3.4% for angiography, 10.7% for ultrasonography, and 39% for radioactive iodine-labeled metaiodobenzylguanidine scintigraphy. Of 192 patients (81.4%) with follow-up data (mean, 43.9 months; range, 0.5-240), operative cure was achieved in 133 (69%). Of the 59 patients without cure, 23 had persistent disease, 5 had recurrent disease, 16 had multiple persistent synchronous tumors, and 15 subsequently developed metachronous tumors. In conclusion, most paragangliomas are nonhypersecretory and located in the head and neck region. Magnetic resonance imaging was associated with the lowest false negative rate, and metaiodobenzylguanidine was the least sensitive imaging study. A significant proportion of patients (31%) has persistent or recurrent disease, and long-term follow-up is important.
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PMID:Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. 1170 78


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