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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pheochromocytoma is a rare tumor that is found in only 0.1% of patients with diastolic hypertension. We analyze the results of our experience in management of pheochromocytomas and long-term results of its surgical treatment. From 1977 to 1996 we operated on 55 patients with a pheochromocytoma: 29 males and 27 females with an average age of 41 years (range 10-63 years). In 44 (80.0%) patients episodic hypertension or paroxysms were observed; 7 (12.7%) patients had permanent hypertension, and 4 (7.3%) had a normal arterial blood pressure (ABP). 131/123I-MIBG scintigraphy (33 patients) and magnetic resonance imaging (12 patients) showed 100% sensitivity and computed tomography (47 patients) 97.9% sensitivity. At operation five (9.1%) tumors were bilateral, five extraadrenal, and five multiple. In four (7.3%) patients an association with familial syndromes (three MEN-IIb, one von Recklinghausen disease) was observed. Five (9.1%) malignant tumors were discovered, and two patients are still alive 30 and 104 months after surgery, one of them with relapse. In 43 (78.2%) patients we preferred a flank incision, and no intraoperative deaths occurred. Mean follow-up was 88 months (6-232 months) with recurrence in only 1 (2.0%) of 50 patients without malignancy. In patients with benign pheochromocytomas the recurrence rate did not seem to be elevated in our series. Nevertheless, because the lifelong follow-up requires only annual 24-hour urinary catecholamine measurement (less than $40 per patient per year) and periodic ABP measurements, it is suggested for all patients who undergo surgery for pheochromocytoma.
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PMID:Pheochromocytoma, a rare cause of hypertension: long-term follow-up of 55 surgically treated patients. 960 83

Presentation of one case of bilateral suprarenal pheochromocytoma as early clinical evidence of a type 2 MEN, which at the time of diagnosis was a bilateral lesion. The presentation form was a picture of shock with HBP. Labetalol i.v. was used for initial control and phenoxybenzamine in the pre-operative. Diagnosis was achieved by biochemical (metanephrine) and radiological (CAT) testing. The patient was managed with bilateral suprarenalectomy through a single, anterior incision and abdominal cavity examination. The investigation of the remaining conditions that conform this syndrome was continued and a familial routine study implemented.
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PMID:[Bilateral adrenal pheochromocytoma]. 965 48

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.
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PMID:The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis. 1006 58

We report the first patient who had bilateral pheochromocytoma associated with multiple endocrine neoplasia type 2a syndrome (MEN 2a) and underwent unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy 2 years later. The postoperative course was uneventful both times, and the patient was cured of hypertension without any need for steroid replacement. Endoscopic partial adrenalectomy is a minimally invasive procedure for pheochromocytoma with mild symptoms. We believe that this procedure has considerable potential for treating bilateral pheochromocytoma, which is frequently observed in patients with MEN 2a.
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PMID:Unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy in a patient with multiple endocrine neoplasia type 2a syndrome. 1021 3

Adrenal medullary hyperplasia is often misdiagnosed. The clinical features may resemble those of pheochromocytoma, with paroxysmal hypertension and elevated urinary catecholamine and metanephrine levels. Pathologic study shows diffuse or nodular adrenal medullary hyperplasia, determined by morphometric analysis: increased adrenal gland weight, increased relative medullary volume, increased relative medullary weight, decreased cortico-medullary ratio. Adrenal medullary hyperplasia may be primary or sporadic, but is often associated with multiple endocrine neoplasia (MEN) type II.
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PMID:[Adrenal medullary hyperplasia: a rare etiology of arterial hypertension--report of a case]. 1032 Sep 11

A case of unilateral adrenal medullary hyperplasia is presented in a 49-year-old caucasian female without multiple endocrine neoplasia association. The patient presented with episodic hypertension and paroxysms suggesting an underlying phaeochromocytoma. Biochemical supported this diagnosis but no discrete tumour was found on preoperative localising studies or at the time of surgery. The patient underwent a unilateral adrenalectomy with confirming adrenal medullary hyperplasia with complete resolution of her symptoms for six months.
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PMID:Unilateral adrenal medullary hyperplasia: another form of curable hypertension? 1034 54

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.
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PMID:Noncardiogenic pulmonary edema as the chief manifestation of a pheochromocytoma: a case report of MEN 2A with pedigree analysis of the RET proto-oncogene. 1052 79

Neoplastic and hyperplastic disorders that affect multiple endocrine tissues in a single individual are well described in humans but less so in domestic animals. Multiple endocrine neoplasia (MEN) in humans is a genetically determined syndrome characterized by the appearance of benign or malignant proliferations within two or more endocrine glands. The primary endocrine tumors that are characteristic of MEN arise from cells that share the capacity for amine precursor uptake and decarboxylation. Here we describe the case of a 22-year-old Thoroughbred mare that died during an unattended parturition and subsequently was presented for necropsy at the University of California, Davis, Veterinary Medical Teaching Hospital. A C-cell (medullary) thyroid adenoma, pheochromocytoma, and multicentric bilateral nodular hyperplasia of the adrenal medulla were present, findings that are remarkably similar to those of human MEN syndrome. Mortality during pregnancy in women with undiagnosed pheochromocytoma is high (approximately 50%), typically because of hypertension and/or hemorrhage associated with catecholamine release from the tumor. Similarly, the mare in this report died of hemorrhage subsequent to parturition. A retrospective evaluation of endocrine tumors in horses that underwent necropsy at the Veterinary Medical Teaching Hospital from 1987 to 1997 was undertaken to identify additional possible cases of MEN in horses. Data from this retrospective evaluation suggest that coexistence of hyperplasias and neoplasias of the thyroid and adrenal glands, similar to MEN syndrome of humans, also occurs with some frequency in the horse.
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PMID:Simultaneous occurrence of multiple neoplasms and hyperplasias in the adrenal and thyroid gland of the horse resembling multiple endocrine neoplasia syndrome: case report and retrospective identification of additional cases. 1056 52

The preoperative evaluation and safe anesthetic treatment of patients with endocrine gland tumors mandate an understanding of the physiologic dysfunctions attributable to these tumors. Some patients will exhibit various signs and symptoms characteristic of the MEN syndromes. In the patient with acromegaly, a fiberoptic-guided intubation of the trachea to secure the airway before induction of general anesthesia must be anticipated. Anesthetic treatment of the patient with hyperadrenocorticism requires knowledge of the physiologic effect of excess cortisol. In the patient with severe hyperparathyroidism, we attempt to correct the markedly elevated plasma calcium levels and maintain adequate hydration and urine output perioperatively. Following thyroidectomy for MCT, 2 potential problems of concern are upper airway obstruction and aspiration resulting from injury (unilateral or bilateral) to the recurrent laryngeal nerve and the superior laryngeal nerve, respectively. The major focus during excision of an insulinoma is prevention of wide swings in blood glucose concentrations. In the gastrinoma patient, the anesthesiologist not only must correct any intravascular fluid volume deficit or electrolyte imbalance but must also consider the patient to have a full stomach at the time of anesthetic induction. Correction of hypokalemia and control of hypertension may be required in the preoperative preparation of the patient with an adrenal cortex tumor. Preoperative alpha-adrenergic blockade must be initiated in the patient with a pheochromocytoma to prevent dangerous elevations in blood pressure during anesthesia and surgery for the tumor's removal. Vasodilators with rapid onset and short duration are used to treat intraoperative hypertension. After ligation of the tumor's blood supply, falls in blood pressure may require treatment with fluids and vasopressors. Carcinoid syndrome patients should be treated with somatostatin to prevent stimuli such as anxiety, abdominal scrubbing, or tumor manipulation from precipitating severe hypotension, hypertension, bronchospasm, or tachycardia. In both pheochromocytoma and carcinoid patients, a smooth anesthetic induction and tracheal intubation plus avoidance of drugs that release histamine or activate the sympathetic nervous system may also prevent intraoperative crises.
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PMID:Anesthetic implications for surgical patients with endocrine tumors. 1081 14

To our knowledge, present case is the first published report of temporal bone findings in multiple endocrine neoplasia type 2B (MEN-2B). We describe a 43-year-old Japanese man with medullary thyroid carcinoma (MTC), pheochromocytoma, mucosal neuroma and a Marfanoid body habitus. The collateral adrenal tumors and MTC were removed surgically. However, 14 years after surgery, the MTC and pheochromocytoma recurred and the patient died of intracranial hemorrhage due to hypertension. During the autopsy, metastatic MTC was detected in the liver, lungs, kidneys, pancreas and cervical lymph nodes. Recurrent pheochromocytoma was present in the right kidney. Mucosal neuromas were found in the tongue, gastrointestinal tract and vesical nerve plexus. The following histopathological findings were seen in both temporal bones: metastatic MTC was found as well as neuromas and the cochlear aqueduct was widely patent.
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PMID:Temporal bone findings in multiple endocrine neoplasia type 2B. 1092 42


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