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)

Therapy of cardiomyopathy is usually symptomatic. So, the etiology is often only superficially investigated. However, because of their curability, rare causes must be sought for. We report a case of acute cardiac failure in a young female patient with severe hypertension of recent onset. Urinary catecholamines analysis and medical imaging demonstrated a paraaortic paraganglioma. After coelioscopic resection, cardiac function recovered.
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PMID:[Acute cardiac failure revealing a paraganglioma]. 1178 87

This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paraganglioma (predominantly mediastinal), or pulmonary chondroma. The authors conducted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypically with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is the second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gastric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney's triad is a rare differential diagnosis for "idiopathic" hypertension or iron-deficiency anemia from chronic gastrointestinal bleeding. If missed, patients with Carney's triad may have the debilitating physical and mental consequences of chronic iron deficiency and may die of untreated prolonged hypertension and metastatic leiomyosarcoma.
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PMID:Typical and atypical Carney's triad presenting with malignant hypertension and papilledema. 1187 80

We describe the successful laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl. A 47-year-old man with hypertension and diabetes mellitus was found to have an abdominal mass beside the aorta. The tumor was diagnosed as a functional paraganglioma by diagnostic imaging and biochemical tests. We then performed a transperitoneal laparoscopic resection for removal. After freeing the left ureter, resecting the inferior mesenteric artery, and dividing the small blood vessels, the tumor was isolated and found to be preserved in its capsule. It was retrieved in a bag through an enlarged incision. The operation time was 450 min and blood loss was 410 ml. The postoperative course was uneventful and there has been no local recurrence or distant metastasis during the 18-month follow-up period. Laparoscopic resection of functional extraadrenal paragangliomas is technically feasible and safe if adequate pre- and intraoperative medical management and a careful, steady surgical technique are used.
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PMID:Laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl. 1196 57

Reports on the association of papillary thyroid carcinoma with paraganglionic or desmoid tumors have appeared infrequently. The former setting usually affects middle-aged females; the latter is typical of familial adenomatous polyposis. We report the case of a 69-yr-old man in whom two abdominal masses had been instrumentally detected following an access of abdominal pain. Save for a moderate hypertension, he was asymptomatic and an impalpable thyroid nodule was detected by ultrasonography. A high urinary noradrenaline output and cytology of the masses raised the suspicion of pheochromocytoma. At laparotomy, an adrenal pheochromocytoma and a paracaval paraganglioma were excised. Subsequently, hemithyroidectomy was performed, and histopathology revealed papillary microcarcinoma. A nodule of desmoid tumor was also removed from the abdominal wall. An analysis of RET, APC, and TP53 gene mutations, and of RET and NTRK1 gene rearrangements, yielded negative results. No in vitro transforming activity was detected in the tumor DNA when assayed in transfection experiments. The lack of a consistent family history also made unlikely the possibility of identifying the putative germline defect by linkage analyses. Should this unusual aggregation of tumors represent a new entity, a number of genetic alterations have now been excluded.
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PMID:Concurrent Pheochromocytoma, Paraganglioma, Papillary Thyroid Carcinoma, and Desmoid Tumor: A Case Report with Analyses at the Molecular Level. 1211 65

Pheochromocytoma is a rare tumour that synthesis extensive amounts of cathecholamines. It is usually called a "ten percent" tumour, as it is inherited in nearly 10% of cases, extra-adrenal in 10%, bilateral, multicentric and malignant in also nearly 10% of cases. Nowadays, surgery of pheochromocytoma is very successful as a result of well equipped laboratory techniques, radiological diagnosis and pharmacological drugs in preoperative therapy and control examinations during surgery. Although the operative technique and anaesthesia are delicate, operative risk is reduced to minimum, and definite recovery can be achieved in almost all benign diagnosed pheochromocytomas. Unrecognized pheochromocytomas remain the main problem, often ending with sudden death because of cerebrovascular complications or myocardial infarction, and are diagnosed during autopsy. The aim of the study is to underline the principal characteristics of pheochromocytomas and evaluate the facts that influence the diagnosis and outcome of the treatment. Medical records of the patients operated on for pheochromocytoma in our Institution are analysed. From 1981 to 2001, 102 patients underwent surgery for pheochromocytoma, 71 females and 31 males, average age 42 years (range 9 to 71). Laboratory confirmation was based on 24-hour urine cathecholamines, and in 12 patients only plasma cathecholamines were determined. In imaging studies, we used ultrasound (US), computerised tomography (CT) and nuclear magnetic resonance (NMR). If there was a suspicion of inherited or extra adrenal form of a tumour, we also used Metaiodbenzilguanidine scan marked with radioactive I131 (I131-MIBG). Diagnostic confirmation of tumours localisation was completely reliable. In 89 patients adrenal pheochromocytoma was found (12 bilateral, 2 with paraganglioma). In 13 patients, pheochromocytoma was extra-adrenal. Inheritated character was registered in 18 patients (MEN2 in 16, NF-1 in 2). Malignant pheohromocytoma was found in 11 patients, in 4 of 13 with extra-adrenal tumours, and in 7 of 89 with adrenal tumours. We prepared all patients with diagnosed pheochromocytoma for operation with phenoxybenzamine in daily doses of 20 to 90 mg, no matter whether they were normotensive or with hypertension. Two days before operation, we included propranolol in the therapy in doses of 20 mg twice or three times a day. Surgery was done in neuroleptic anaesthesia. We performed 100 primary operations and 9 reoperations (in 2 patients, the first operation was carried out in another institution), and extracted 123 tumours. In 2 patients, during reoperation we extracted the opposite adrenal tumour, in one recurrent benign tumour, and in the others, reoperation was done because of recurrent malignant pheochromocytoma. Hypertension was completely corrected in 79% of operated patients, reduced in 13%, and in 7% there was no effect on hypertension We observed the following operative complications: peritonitis due to a small bowel lesion (1), infection and wound dehiscence (1), pleural effusion (1), pneumothorax (1), gastrointestinal haemorrhage (1), haemathoma (1), temporary hemiparesis (1) and seroma of the wound (1). There was no operative mortality.
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PMID:[Results of surgical treatment of pheochromocytoma at the Institute of Endocrinology of the Clinical Center of Serbia in Belgrade]. 1258 96

Pheochromocytoma and paraganglioma are neoplasias of the chromaffin cells that manifest themselves in some 40-60% of cases by symptoms of episodic freeing of catecholamines. They are the cause of stable and malign HTA in some 0.1-1% of cases and must be discounted in a study of the same. They most frequently affect males and their incidence increases with age. The symptomatic episodes are basically characterised by high blood pressure, migraines, sweating and palpitations, although they can have other multiple manifestations and even take a silent form in upto 40-50% of cases. They are frequently associated (10%) with multiple endocrine neoplasias and other neuroectodermic syndromes. For their diagnosis it is essential to measure the catecholamines and their metabolites, both plasmatic and urinary, and to locate the tumour by means of image tests, especially magnetic resonance. In the handling of these tumours it must be born in mind that the carrying out of physical maneuvers on the tumour and the employment of drugs that free catecholamines can induce hypertensive crises. The treatment chosen is always surgical, curative in cases of benign pheochromocytoma or producing a reduction of the tumourous mass and associated symptoms in cases of malign pheochromocytoma (10%); in this latter case treatment is complemented with chemotherapy or drugs that control the tumourous symptoms. Untreated pheochromocytoma can be a mortal disease, hence the importance of its detection and early treatment.
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PMID:[Pheochromocytoma and paraganglioma]. 1289 19

The authors present a case of a 37 year old male (proband) with a 13 year history of progressive sight impairment leading to blindness and a 4 year history of a mild hypertension. He was incidentally found to have large adrenal tumors after an ultrasound kidney examination. The tumors were confirmed with CT scan and magnetic resonance imaging. A bilateral pheochromocytoma was biochemically demonstrated and successfully removed. The eye diagnosis of angiomatosis retinae von Hippel-Lindau was ascertained after a search of the patient files in other medical departments, which led to a family screening. Proband's brother, having hypertension and a history of a cerebellar astrocytoma operation, was also diagnosed with CT scan to have a bilateral pheochromocytoma. Unfortunately, at the same time he was found to have a large irremovable neuroendocrine pancreatic carcinoma, which caused complications and his eventual death. Both proband and his brother were affected by the same CGG(Arg167)->CAG(Gln) mutation in the exon 3 of the VHL gene. Other living and examined family members were not affected, which was confirmed by negative genetic testing. One year after the pheochromocytoma operation, proband was diagnosed to have a retroperitoneal tumor left to the aorta, clinically silent, with slightly and non-constantly elevated urine norepinephrine and normetanephrine. Metaiodobenzylguanidine scintigraphy showed that it was a paraganglioma. The old CT and magnetic resonance picture review demonstrated that the tumor had already been present at the time of the operation. It was surgically removed and histologically verified. It is a pity that proband had not been sent by his ophthalmologist for an endocrine examination when the eye diagnosis was determined. Affection of the family would have been discovered earlier, and proband's brother might have possibly been saved.
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PMID:[Hereditary pheochromocytoma--a family affected by von Hippel-Lindau disease]. 1293 43

Pheochromocytoma is a rare tumor, but it represents a potentially curable form of hypertension. In patients with inherited pheochromocytoma, benign and bilateral tumors are more common. The diagnosis of pheochromocytoma rests in biochemical confirmation of catecholamine excess. Plasma-free metanephrine levels are arguably the most sensitive and specific test for the biochemical diagnosis of pheochromocytoma in high-risk patient populations. A timed 24-hour urine collection for total catecholamines and metabolic products (eg, vanillylmandelic acid and metanephrines) is the favored confirmatory test. Localization is most commonly accomplished with high-resolution computed tomography imaging, but magnetic resonance imaging can also be used. If both of these imaging modalities are nonlocalizing or equivocal, then radiolabeled meta-iodobenzylguanidine or somatostatin can be used to identify an adrenal or extra-adrenal tumor (paraganglioma). These imaging modalities can be used in the evaluation of patients with suspected or confirmed recurrent or metastatic disease. Systemic therapies for the treatment of patients with recurrent or metastatic disease have been disappointing. Radiation therapy is best applied for palliative relief of pain associated with bony metastases. In the absence of radiographic evidence for local tumor invasion, laparoscopic resection of small- to medium-sized (< 6 cm) pheochromocytomas is indicated. Abundant evidence indicates that this approach is safe and well tolerated and results in more rapid recovery and less long-term wound morbidity compared to open anterior or posterior adrenalectomy. Open anterior adrenalectomy is appropriate for patients with large or recurrent tumors, suspected or documented locoregional invasion, or for those patients in whom a laparoscopic approach is technically contraindicated. For selected patients with pheochromocytoma in the von Hipple-Lindau syndrome or multiple endocrine neoplasia type 2 setting in which the cumulative incidence of clinical bilateral tumors is high, a cortical-sparing approach may minimize the risk of Addisonian complications.
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PMID:Pheochromocytoma. 1294 13

We report a potential pitfall of 123I-metaiodobenzylguanine (MIBG) scan. Magnetic resonance imaging performed for other reasons, showed 2.5 cm tumor in the left adrenal gland. On questioning patient had episodic palpitations, flushing and hypertension suggestive of pheochromocytoma. Urinary metanephrine level was of borderline value but serum chromogranin A level was clearly elevated. 123I-MIBG scan showed accumulation of the tracer in the upper left abdomen and the finding was suspected to be intra-adrenal pheochromocytoma. During operation two separate tumors, adrenocortical adenoma in the left adrenal gland, and another smaller, extra-adrenal paraganglioma locating very close to the adenoma, were found. Thus the positive MIBG finding was caused by a paraganglioma with the concurrent presentation of nonfunctioning adrenocortical adenoma.
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PMID:A pitfall of metaiodobenzylguanidine scan: paraganglioma in close proximity to adrenocortical adenoma. 1295 37

Paragangliomas of the head and neck are uncommon neoplasms. They are usually benign, but tend to be locally invasive. Although surgical resection remains the definitive treatment, important issues about management arise when such lesions are inoperable. Beneficial effects of octreotide treatment have already been reported in a malign paraganglioma case. Here we report a 24 year old female with familial, bilateral, multiple paraganglioma in the head and neck region, who firstly presented with pulsatile tinnitus and hearing loss in her left ear. After embolization was performed, she underwent operation twice because of the gross tumor mass. No significant change in tumor size was determined after the operations, however there were no distant metastases. Although she experienced hypertension attacks, no hormonal overproduction was found in repeated measurements. As the tumor was unresectable, new alternative therapies were sought. Octreotide scintigraphy was positive in the tumoral tissue, so we began to treat her with somatostatin analogue octreotide. After a 16 month follow up period, an improvement of the performance status, the near normalisation of attacks and stabilization of tumor growth were achieved. However, in the last three visits, she began to experience symptoms more frequently and it had been necessary to increase the octreotide dose. She is now well and being followed up. In conclusion, the beneficial effects of octreotide treatment could be quantified by clinical, tumor and scintigraphic criteria. These data suggest that octreotide can be useful in the treatment of inoperable paragangliomas.
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PMID:Case report: patient with multiple paragangliomas treated with long acting somatostatin analogue. 1461 6


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