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

Pheochromocytoma remains a clinical challenge to diagnose and manage. In addition, the association of multiple endocrine neoplasia syndromes with pheochromocytoma require the clinician's awareness to evaluate patients with pheochromocytoma (especially when bilateral) for abnormalities in thyroidal C-cell function with serum calcitonin determinations. The authors present a case of a 42-year-old woman initially diagnosed with, and treated for, cranial artery vasculitis because she had a stroke and a history of rheumatoid arthritis and asthma. Subsequent evaluation of episodic blood pressure increases, headache, and tachycardia revealed biochemical evidence of catecholamine overproduction. Bilateral adrenal masses were found on computed tomography scanning, and the functional nature of the adrenal masses was confirmed by a meta-Iodobenzylguanidine scan. Upon further evaluation, an elevated serum calcitonin concentration was demonstrated, which increased greatly with pentagastrin stimulation. C-cell hyperplasia was demonstrated by subsequent thyroidectomy, confirming the diagnosis of multiple endocrine neoplasia 2A. The difficulty in arriving at a correct diagnosis, the subsequent management, including bilateral adrenalectomy and thyroidectomy, and newer insight into the genetic abnormalities of multiple endocrine neoplasia 2A are discussed.
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PMID:Case report: bilateral adrenal pheochromocytoma. 804 54

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

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 58 year old woman had a long history of immunocompromised state. Since age 28 she had multiple endocrine neoplasm type 2A: her thyroid gland and bilateral adrenal glands were removed because of pheochromocytoma and thyroid medullary carcinoma. Corticosteroid and levothyroxine were supplemented. At age 57 she was afflicted with systemic lupus erythematodes and nephrotic syndrome. Prednisolone therapy was started. Two months later she developed fever, lethergy, headache and left hemiparesis. MRI revealed multiple ring-enhancing lesions in the right cerebrum. CSF was negative for microorganisms. Blood culture hemolysed after 24 hours. Direct gram staining of the blood culture sample revealed gram-positive short rods without spore, suggested listeria. This enabled prompt initiation of high dose penicillin therapy before the official report of listseria infection. Neurological abnormality including left hemiparesis disappeared completely within one month. Enhancement of abscess wall decreased every month, but it persisted for five months despite continuous intravenous penicillin therapy. Listeria monocytogenes is well-recognized as an opportunistic pathogen. It requires prolonged therapy with antibiotics, since it is the intracellular organism. Monitoring of the brain abscess wall by the enhanced MRI is useful to determine the completion of therapy. Since listerial contamination is common among raw meat and unpasteurized milk, immunocompromised patients should be alarmed not to eat uncooked food products.
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PMID:[Direct Gram staining of blood culture sample enabled the early diagnosis of brain abscess due to Listeria monocytogenes]. 1068 44

We report a case of synchronous bilateral pituitary adenomas in a patient with multiple endocrine neoplasia type one (MEN1). The patient was previously known to have a pancreatic gastrinoma and had first-degree relatives with MEN1. Both adenomas were concurrently revealed by high-resolution MR imaging of the pituitary gland as part of the investigation of the patient's severe, persistent headaches and elevated serum prolactin level.
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PMID:Bilateral pituitary adenomas occurring with multiple endocrine neoplasia type one. 1087 Oct 15

Pheochromocytoma is a catecholamine-producing tumor which can be life-threatening. A series of 40 operations in 39 pheochromocytoma patients at a tertiary hospital in Thailand from 1976 to 1997 was reported. The patients were 30 females and 9 males; aged 7-73 years. One man had 2 operations 5 years apart. The most common symptoms and signs were palpitation, headache and hypertension. Preoperative management consisted of control of blood pressure and restoration of intravascular volume by using prazosin, an alpha adrenergic blocker. New imaging techniques have improved the ability to localize the tumors; 20 were found in the right adrenal glands, 14 in the left, 1 patient had bilateral tumors, 4 in Organs of Zuckerkandl and 1 patient had metastatic liver nodules. The operative procedures were 39 laparotomies and 1 laparoscopic surgery. The surgical and anaesthetic procedures were presented, and nitroprusside was used to control intraoperative blood pressure. Removal of tumors was successful in all cases except for 1 mortality due to injury of the liver and massive blood loss. Other complications were postoperative pulmonary edema and renal vein thrombosis. One patient had MEN type 2 and five cases were malignant. Pheochromocytoma can be cured by surgery, but cooperation among surgeons, anesthesiologists and internists is very important.
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PMID:Surgery and anesthesia for pheochromocytoma--a series of 40 operations. 1099 47

MEN-1 is an autosomal dominantly inherited disorder, characterised by the occurrence of multiple tumours, particularly in the parathyroid glands, the pancreatic islets, the pituitary gland and the adrenal glands, as well as by neuroendocrine carcinoid tumours. Various clinical manifestations are presented by description of three patients harbouring a MEN1 gene germline mutation. A 44-year-old man had symptoms of hyperparathyroidism and in addition to parathyroid adenomas proved to have tumours in the thymus, adrenal and pituitary glands. A 48-year-old woman from a family with MEN-1 had suffered since her 40th year from headache and heartburn; she appeared to have adenomas in the parathyroid glands and gastrinomas in the pancreas, leading to a Zollinger-Ellison syndrome. One of her relatives, a man aged 29, had suffered from childhood from convulsions due to attacks of hypoglycaemia, and an insulinoma was assessed. In all patients, surgical and/or medical treatment alleviated symptoms. Clearly, the position or nature of the mutations in the MEN1 gene do not correlate with the clinical expression of the disease. Family investigation, DNA analysis and periodic examination improve quality of life and the life expectancy.
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PMID:[Diverse expression of multiple endocrine neoplasia type 1]. 1115 52

In a 25-year-old man, medullary thyroid carcinoma (probably a solitary sporadic form) was diagnosed following investigation of a small lump in the patient's neck. This was removed and followed up with further treatment. In a 27-year-old man, episodes of headache, palpitations and excessive perspiration (due to a pheochromocytoma) and a positive family history of thyroid problems led to further investigations and the subsequent diagnosis of multiple endocrine neoplasia (MEN) type 2A. The patient died at 48 years of age as the result of liver metastases. A total thyroidectomy had been carried out on a 19-year-old man with familial medullary thyroid carcinoma. The calcitonin levels remained elevated, but no tumour residues could be found. A 16-year-old girl with MEN type 2B had also previously undergone surgery. Her main complaint consisted of persistent constipation. Thyroid carcinomas usually present as a nodule in the neck. In 25% of cases, medullary thyroid carcinoma is part of the MEN2 syndrome. The clinical approach for medullary thyroid carcinoma is based on pathological findings following fine needle aspiration biopsy. The results of DNA tests determine the course of treatment and the need for family testing. In families with a hereditary form, there is a clear genotype-phenotype correlation. Early diagnosis and treatment can improve life expectancy.
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PMID:[Lump in the neck: 4 patients with a medullary thyroid carcinoma]. 1175 44

Phaeochromocytoma is a tumour of the adrenal medulla, which, although rare, is a major cause of correctable hypertension with a prevalence of 0.1-0.5% in the hypertensive population. Clinical symptoms include attacks of paroxysmal headache, sweating, palpitations, stress and a sense of imminent death. Often associated with the above is an increase in blood pressure. Despite the fact that the underlying genetic mechanisms of phaeochromocytoma have been well investigated, they are still incompletely understood. In approximately 80% of cases the tumour occurs sporadically, but it may occur in association with type 2 multiple endocrine neoplasia, type 1 neurofibromatosis or von Hippel-Lindau disease. Molecular evidence suggests that other genes such as SDHD or SDHB may control its development; the possibility of other putative phaeochromocytoma genes is currently being investigated.
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PMID:Clinical and genetic aspects of phaeochromocytoma. 1256 22


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