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Query: UMLS:C0018681 (headache)
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Fourteen children (10 boys and 4 girls, aged 8 to 17 years) had 20 pheochromocytomas treated over a 36-year period from 1959 to 1995 inclusive. Nine patients had 11 tumors before 1980; 5 children had 9 tumors up to 1987. There were no new children with pheochromocytomas at our hospital from 1988 to 1995. Hypertension, sweating, headache, and visual blurring were the most common symptoms and signs (average 5 months). The most reliable biochemical investigations were the urinary catecholamines and norepinephrine. Before 1980, intravenous pyelography and angiography were most successful in localizing the tumor, but since then ultrasonography and computerized tomography have been the radiological investigations of choice. Early involvement of the anesthesiologist in the preoperative control of the hypertension is essential; blood pressure (BP) control was achieved with phenoxybenzamine. The main anesthetic drugs used were: sodium thiopental, fentanyl, methoxyflurane, isoflurane, nitrous oxide, and metocurine. Sixteen tumors were adrenal and 4 were extra-adrenal (1 intrathoracic and 1 extradural). All except 2 tumors were completely resected; they ranged in size from 1.3 to 14 cm. Ligation of the tumor's venous drainage was usually associated with a sudden, temporary fall in systemic BP. There were 2 children with malignant tumors. Four patients had five recurrences (second pheochromocytoma) within 6 years, and all were heralded by a return of their original symptoms and signs. One girl was left with no adrenal tissue. The only complication was in a boy with a large, partly-resected malignant right adrenal tumor who had a subphrenic abscess drained and was left with a temporary bile fistula, cirrhosis, and chronic pain. All children were normotensive when discharged from hospital and remain alive and well with a follow-up of 7 to 36 years. There were no deaths. Long-term follow-up is essential. Key word Pheochromocytoma
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PMID:Pediatric pheochromocytoma. A 36-year review. 935 33

We experienced a case of MEN type 2a with bilateral and large pheochromocytomas. A 39-year-old man was admitted to the previous hospital with complaints of paroxysmal headache. hypertension and diabetes mellitus. Radiographic imagings showed thyroid tumors in both lobes and bilateral adrenal tumors. Thyroid tumors were histologically proved to be medullary thyroid carcinoma by needle biopsy and systemic investigations revealed an excessive secretion of plasma and urinary cathecholamines which suggested the presence of pheochromocytoma. The patient was diagnosed as MEN type 2a. He was admitted to our hospital for the treatment of bilateral adrenal tumors for which we performed one-stage bilateral adrenalectomy by thoracoabdmonal approach. Both adrenal tumors were histologically confirmed as pheochromocytoma. The patient's postoperative course was uneventful. He underwent uneventful total thyroidectomy approximately 2 months after bilateral adrenalectomy. Even in bilateral and large pheochromocytomas, one-stage bilateral adrenarectomyenables safe postoperative managements. We concluded that the thoracoabdominal approach is feasible in the patients with huge and cranially spreading adrenal tumor, which gives us a wide operative field for easy vascular control.
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PMID:[One-stage-bilateral adrenalectomy by thoracoabdominal approach for bilateral large pheochromocytomas in men type IIa. A case report]. 938 71

A 53 years old man had an angiography for suspected renovascular hypertension (arteritis, renal insufficiency, duplex scanning). It showed a narrow right renal artery streched by a 45 mm mass arising from the adrenal. The computed tomography showed the tumor and the nuclear magnetic resonance imaging indicated a pheochromocytoma. The patient had no complain of headaches, palpitations or sweating. Biochemistry was normal except for a slight serum creatinin elevation and a non significant urinary noradrenaline level. A diagnostic of non functioning pheochromocytoma was made. The therapeutics consisted in a surgical ablation of the tumor and the right kidney (non functioning) and the patient became normotensive thereafter without treatment. The histologic feature was an aortico-sympathetic paraganglia, the adrenal was normal. Paraganglias are arising from the paraganglion system including chemodectoma and glomus jugulare tumor. Non functioning retroperitoneal paraganglias are uncommon: less than 50 in the literature between 1902 and 1992.
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PMID:[Para-aortic paraganglioma found by arteriography. Apropos of a case and review of the literature]. 940 33

The case of secondary hypertension to pheochromocytoma in 71 year old woman is described. This woman has been made a false diagnosis of primary hypertension 10 years earlier. The primary objective of this paper was to call practitioners' attention to the necessity of penetrating diagnosis of arterial hypertension, also in elderly patients. Over-hasty diagnosis of primary arterial hypertension hampers the possibility of proper and causal treatment. Labile and paroxysmal hypertension with headache, tachycardia and sweating should alert the clinician to tumor of the adrenal medulla as well as the extra-adrenal paraganglion system (pheochromocytoma is the cause of arterial hypertension in 0.1-1.0% of all cases). Twenty four hours excretion level of vanillylmandelic acid in urine is of diagnostic relevance for patients with pheochromocytoma. The measurement which is done after elevation of blood pressure is more sensitive.
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PMID:[Secondary hypertension during a course of phaeochromocytoma in a 71-year old woman]. 943 94

A 40-year-old woman was admitted because of abdominal pain and diarrhea. She sometimes experienced paroxysmal hypertension, sweating, headache, and palpitation. Sigmoidoscopic findings showed well-demarcated diffuse mucosal edema, hyperemia, and easy touch bleeding from distal descending colon up to the splenic flexure area. Barium x-ray showed loss of haustral marking, thumb printing appearance, and diffuse luminal stenosis in the transverse, descending, and sigmoid colon. On the abdominal computed tomogram, a 3.8-cm sized well-enhanced right adrenal mass was incidentally found. Twenty-four hour urinary excretion of vanillyl mandelic acid, norepinephrine, and normetanephrine were increased. Iodine131 metaiodobenzylguanidine scan showed hot uptake on the right adrenal gland compatible with pheochromocytoma. Exploratory laparotomy was done under the impression of ischemic colitis associated with pheochromocytoma. Adrenalectomy and resection of the stenotic left colon were performed. After surgery, pain subsided, blood pressure fell gradually, blood sugar and catecholamine level became normal, and bowel habit returned to normal.
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PMID:A case of ischemic colitis associated with pheochromocytoma. 944 95

A 57-year-old woman complaining of hypertensive attacks associated with headache and palpitation was admitted to our hospital for examination of bilateral adrenal tumors in 1985. After right pheochromocytoma was diagnosed, right adrenalectomy was performed. The left adrenal tumor was small; thus, the left adrenal tumor was preserved to avoid adrenal insufficiency, although left adrenal pheochromocytoma could not be denied. In 1995, she was referred to our clinic again because of recurrent headache and palpitation, and enlargement of the left adrenal tumor. Although pheochromocytoma was suspected again, the enlarged left adrenal turned out to be a nonfunctioning cortical adenoma. This case was interesting, not only because of difficulty in identifying the left adrenal tumor, but also because of the rare coexistence of a pheochromocytoma and a nonfunctioning adrenal cortical tumor.
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PMID:Unique association of pheochromocytoma with contralateral nonfunctioning adrenal cortical adenoma. 950 59

Headache is an important diagnostic element in pheochromocytoma and it may characterize a body reaction to pathological hormonal oscillations. We observed the pheochromocytoma instability in 20 patients during arterial hypertension and tried to correlate with headache. We found that isolate hypertension is not the only factor in headache pathogenesis. It is possible that changes in catecholamines, adrenomedullin and other neuropeptides may cause some of these symptoms.
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PMID:[Headache in patients with pheochromocytoma. Influence of arterial hypertension]. 969 37

We report a case of pheochromocytoma with a markedly large pseudocyst. A 51-year-old man with the complaint of headache and palpitation was referred to our hospital. Ultrasonography (US) revealed a right adrenal cystic mass. Computerized tomography (CT) and magnetic resonance imaging (MRI) confirmed an adrenal cyst. MRI showed a high intensity mass in both T1 and T2-weighted-images, suggesting an adrenal hemorrhagic cyst. 131I-meta-iodo-benzylguanidine (MIBG) scintigraphy showed a prominent accumulation at the right adrenal lesion. Considering the abnormal elevation of cathecholamines in blood and urine samples, we performed right adrenolectomy with the clinical diagnosis of pheochromocytoma. 131I-MIBG scintigraphy was the most important diagnostic tool in the diagnosis of pheochromocytoma.
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PMID:[A case of pheochromocytoma with a large pseudocyst]. 971 39

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

Pheochromocytoma usually presents with nonspecific symptoms such as headache, palpitation, chest pain and hypertension. We present a 48-year-old woman hospitalized due to a pheochromocytoma multi-organ crisis (PMC). It consists of a tetrad of symptoms: multi-organ failure, hyperthermia, encephalopathy and unstable blood pressure. Distinguishing PMC from septic shock may be difficult, and requires needs a high clinical index of suspicion.
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PMID:[Pheochromocytoma multi-organ crisis: nonseptic hyperthermia and shock]. 988 52


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