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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the past 20 years (1970-90), we had 24 patients with pheochromocytoma: 19 diagnosed clinically and 5 post-mortem. Their ages ranged from 17 to 74 (mean, 43.2 years). Males (n = 14) outnumbered females (n = 10), a 1.41:1 M:F ratio. A majority were symptomatic (95%), with a typical triad of headaches, palpitations and diaphoresis. Most frequent finding was hypertension (95%). It was sustained in 60% and paroxysmal in 35%. In 6 patients (25%) pheochromocytomas were bilateral, all familial. Fifteen were solitary adrenal tumors (63%); 3 (12.5%) were extra-adrenal: 2 intra-abdominal, and 1 cardiac paraganglioma of right atrium. Of 6 familial cases, 4 were associated to Von Hippel-Lindau (VHL) disease, while 2 were multiple endocrine neoplasia (MEN-II) patients. All familial cases were bilateral and in the adrenals. There were no malignancies. Among the 19 clinical cases pre-operative Dx was made by positive urine VMA or catecholamines urine levels: (95 and 100% sensitivity respectively). Preoperative visualization by CT or MRI was done in 62% of the most recent patients. In 5 earlier cases the diagnosis was made post mortem: 3 died of cerebral hemorrhage, 1 with a pons infarct and 1 with congestive heart failure (CHF). There were 2 post-operative deaths and another died 13 years later from thyroid medullary carcinoma. Of the 19 operated, 13 (68%) were cured. Thus pheochromocytomas retain considerable morbidity and some mortality. These rare tumors constitute a clinical diagnostic challenge yet a rewarding therapeutic experience for the alert physician.
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PMID:Pheochromocytoma: a twenty year experience at the University Hospital. 177 16

A 23-year-old woman with von Hippel-Lindau disease had headaches, visual disturbances, and an enlarged sella. Cerebral angiography demonstrated multiple posterior fossa hemangiomas and obstructive hydrocephalus. Massive dilation of the third ventricle compressed the chiasm, resulting in an unusual cause of visual disturbance.
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PMID:Visual disturbances in von Hippel-Lindau disease. 719 79

Eight cases of pheochromocytoma in children were managed between 1958 and 1978. Headache, visual blurring, sweating, and hypertension were the most common findings. One patient presented with a hypertensive crisis during appendectomy. Three other children had a family history of pheochromocytoma and Hippel-Landau disease, thyroid carcinoma, and renal stones. The diagnostic approach was based on a high suspicion of the disease from the clinical picture. The most reliable laboratory investigations were urinary VMA, metanephrine, and catecholamines. I.V.P. and angiography were successful in localizing the tumor. In 4 cases, CT scan localized the tumor, although a second tumor was not seen in 1 case. Preoperative control of hypertension was achieved with phenoxybenzamine, propanolol, apresoline and reserpine in seven cases. The main anesthetic drugs used were pentothal, methoxyflurane, nitrous oxide, Innovar, and pancuronium. Tumors were located in the right adrenal in four, the left adrenal in two, both adrenals in one, and bilateral para-aortic sites in one. In two cases, hypertensive episodes during manipulation of the tumor were controlled with phentolamine. Ligation of the venous drainage from a tumor was associated with a sudden sustained fall in systemic blood pressure in six cases. In two, blood pressure remained elevated until a second tumor was found and removed. Hypotension was then corrected by the rapid infusion of fluid and blood, and vasopressors were not used. There were no postoperative complications and all children remained normotensive during the follow-up. The tumors of seven of the patients were benign. One tumor showed a low grade malignancy.
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PMID:Pheochromocytoma in children--an update. 741 63

The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel-Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.
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PMID:Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery. Case report. 884 70

We retrospectively evaluated our experience with phaeochromocytoma from January 1986 to December 1995. There were 18 patients with surgically-proven phaeochromocytoma: three males, 15 females, aged 12-81 years (mean 42 years) at diagnosis. Sixteen were hypertensive; only 6/18 presented with two or more of the classical triad of headaches, palpitations and diaphoresis. One patient presented with hypertensive crisis. Duration of symptoms prior to diagnosis was 2 weeks to 6 years, mean 16.4 months. Sixteen patients had adrenal tumours and two had extra-adrenal tumours or paragangliomas. One had bilateral adrenal tumours and two had a combination of both adrenal and extra-adrenal tumours. There were four familial cases: two had multiple endocrine neoplasia type IIA (MEN-IIA), one had neurofibromatosis type I (NF-I) and one von Hippel-Lindau (VHL) disease. One patient had Cushing's syndrome arising from ectopic production of adrenocorticotropic hormone (ACTH) by the phaeochromocytoma. Disease was recurrent in three patients. Pre-operative diagnosis was confirmed mainly by elevated urine vanillylmandelic acid (VMA) and/or catecholamine levels. Twelve patients had plasma catecholamine determinations: noradrenaline was elevated in all, adrenaline in six and dopamine in two. Pre-operative localization was by CT scan or MR imaging in all patients. At follow-up of 1-10 years (median 4.8 years), 15 patients were cured surgically while two were asymptomatic despite recurrence of disease. One patient with recurrent paragangliomas died post-operatively.
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PMID:Phaeochromocytoma: a ten-year survey. 909 89

Pheochromocytomas are catecholamine-producing tumors, representing one of the most important causes of secondary hypertension. The classification of these tumors considers both sporadic and familial forms, intra- and extraadrenal localization as well as the dignity. Familial pheochromocytomas are primarily seen under the conditions of multiple endocrine neoplasia, von Hippel-Lindau disease or neurofibromatosis type 1. The list of clinical symptoms includes hypertension, which can be both continuous or intermittent, headache, tachycardia and sweating. It is most important to standardize the pre-analytical procedures, i.e. control for sampling conditions and adequate choice of parameters in plasma or urine. For screening sensitive methods will be employed (free catecholamines in 24h-urine) and for confirmation of the diagnosis, specific procedures are performed (Clonidine test, MIBG-scintigraphy). The endocrinological and biochemical procedures are completed by molecular genetic techniques in familial pheochromocytoma.
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PMID:[Clinical and endocrine diagnosis of pheochromocytoma]. 933 11

Although adrenal tumors detected during pregnancy are extraordinarily rare, the pathophysiologic repercussions of untreated adrenal neoplasms are enormous to both mother and fetus. From our computer-based registry of pregnant patients from 1975 through 1996 (n = 30,246), four cases of adrenal neoplasms associated with pregnancy were identified (0.013%), analyzed, and compared with the current medical literature. Four women ages 36, 29, 22, and 21 years had adrenal neoplasms diagnosed with pregnancy. Patient 1 had an unsuspected pheochromocytoma identified at autopsy. At 27 weeks into her pregnancy the patient suffered a myocardial infarction, and both she and the fetus died. Patient 2 was incidentally found to have adrenal and pancreatic neoplasms on screening abdominal computed tomography for von Hippel-Lindau disease. The study identified a pregnancy. She elected to terminate the pregnancy and underwent resection of both tumors. She died 3 years later of metastatic islet cell cancer. Both of these patients had previously delivered healthy babies, but both pregnancies were complicated by hypertension. Patient 3 had a functional adrenal tumor identified initially by urinary aldosterone studies because of symptoms of severe hypertension, and patient 4 had an adrenal mass diagnosed via ultrasonography at 30 weeks' gestation because of concerns for right-sided pyelonephritis. These two women underwent careful monitoring throughout the remainder of their pregnancies with eventual delivery of healthy babies. Both women later underwent successful operative resection of benign adrenal adenomas. Adrenal neoplasms discovered during pregnancy are rare. The onus, however, is on physicians to consider this diagnosis in pregnant women with hypertension, headaches, or other manifestations of adrenal disorders. Surgical management of identified adrenal lesions is thereafter straightforward. Missing the diagnosis has grave implications for these young women and their fetuses.
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PMID:Adrenal tumors and pregnancy. 988 Apr 29

We report the clinical, epidemiological and pathological findings of 14 patients with haemangioblastoma. Sixty-four percent occurred in males, with ages ranging from 16 to 60 years, with an average of 34, 4 years. Most of the tumours were confined to the cerebellum (n=9). The most frequent symptoms were headache (n=7) and dizziness (n=7), with a mean duration of 70 days. Von Hippel-Lindau syndrome (vHL) was diagnosed in 3 patients. Eleven patients were submitted to total surgical removal and in 3 the tumour was partially resected. A relapse rate of 28% in 3 years of follow-up was found. The patients with vHL showed recurrence in 66% of the cases. These findings corroborate those in current medical literature, showing an increased morbidity of this tumor when associated with vHL.
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PMID:[Haemangioblastomas: clinical, epidemiological and pathological findings in 14 cases]. 1084 33

The pheochromocytoma is a medullo-adrenal tumor which develops at the cost of the chromaffin cells. It appears in 11-19% of cases of von Hippel-Lindau's disease (VHL), is often bilateral, and the symptomatology is often crude: arterial hypertension is frequently isolated and unstable, and the classic triad of headache, palpitations and sweating is quite rarely observed. We report four observations of bilateral pheochromocytomas in patients with von Hippel-Lindau's disease (three with phenotype IIA and one with phenotype IIB). The tumor was bilateral during the diagnosis in three cases; in the fourth patient, the attack on the contralateral adrenal gland came two years after the first adrenalectomy. All the patients had undergone an adrenalectomy by open surgery after a short preparation of 48 hours; replacement therapy was begun in each patient. Morbidity was low, and the patients submitted to a prolonged follow-up in order to screen for the onset of future lesions of VHL.
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PMID:[Bilateral adrenal pheochromocytomas in von Hippel-Lindau disease]. 1177 64

A rare case of von Hippel-Lindau (VHL) disease with bilateral pheochromocytomas, right renal cell carcinoma, right pelvic carcinoma, spinal hemangioblastoma and primary hyperparathyroidism is described. A 78-year-old woman had a history of hypertension from her forties. She suffered from headache and body weight loss. Abdominal CT revealed bilateral adrenal tumors and right external renal tumors enhanced in early stage. MIBG scintigraphy exhibited a high accumulation of tracer in both adrenal glands. On the basis of the radiographic findings and endocrinological results, the patient was diagnosed as having bilateral pheochromocytomas and right renal cell carcinoma. A bilateral adrenectomy was performed, followed by surgery for resection of the renal cell carcinoma. The other resected right kidney showed a clear cell subtype that was determined to be renal cell carcinoma, and proved that the pelvic tumor was transient cell carcinoma. Spinal MRI showed spinal hemangioblastoma. von Hippel-Lindau (VHL) gene mutation for the patient was found. We diagnosed the patient as VHL because of the existence of spinal hemangioma and a VHL disease gene. Parathyroid echo revealed a hypoechoic space on the back of the left lobe, and serum calcium and intact PTH to be elevated. The patient was diagnosed as primary hyperparathyroidism. We report the first case of a patient with VHL disease complicated with bilateral pheochromocytomas, right renal cell carcinoma, right renal pelvic carcinoma and primary hyperparathyroidism. The life expectancy of affected individuals has been less than 50 years. Since the prognosis may be improved by an early diagnosis, affected individuals with VHL complexes should undergo cranial, spinal MRI and abdomen CT. The families may benefit from presymptomatic detection of affected gene carriers and the exclusion of at-risk family members by negative test results.
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PMID:A case of von Hippel-Lindau disease with bilateral pheochromocytoma, renal cell carcinoma, pelvic tumor, spinal hemangioblastoma and primary hyperparathyroidism. 1208 Dec 37


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