Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Baseline plasma norepinephrine (NE) and epinephrine (E) levels over 2000 pg/ml or failure to suppress to less than 500 pg/ml after oral clonidine have been considered diagnostic of the presence of a pheochromocytoma. We found a false negative clonidine suppression test in a patient with an asymptomatic ACTH-secreting pheochromocytoma who had minimally increased resting plasma NE and E values of 669 and 419 pg/ml, respectively. Clonidine suppression caused decreases at 2 and 3 h to 372 and 408 pg/ml, respectively. A positive test was found in a patient with repeatedly elevated baseline plasma NE and E concentrations; the two highest results were 2501 and 3022 pg/ml. Clonidine administration on five occasions failed to decrease plasma NE and E levels to less than 500 pg/ml. However, no pheochromocytoma was found by selective venous catheterization, two laparotomies, and, ultimately, postmortem examination. Diffuse infiltration of lymphoplasmacytic cells throughout sympathetic ganglia and adrenal medulla raise the possibility of a diffuse autoimmune disorder, resulting in excessive catecholamine production. These examples suggest that the clonidine suppression test does not always indicate the presence or absence of a pheochromocytoma.
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PMID:Clonidine suppression test for pheochromocytoma: examples of misleading results. 371 Dec 61

A 42-year-old female with clinical and endocrine indications of Cushing's syndrome, as well as periodic hypertension and increased urinary catecholamines and their metabolites, benefitted from removal of a pheochromocytoma. Adrenocortical hyperplasia was present. Electron microscopy showed catecholamine-type granules in the tumor cells; in addition, immunoreactive ACTH, leu-enkephalin, somatostatin, and serotonin were identified. Such studies were performed for the first time in this unusual condition.
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PMID:Pheochromocytoma producing immunoreactive ACTH with Cushing's syndrome. 609 99

An autopsy case of a 54-year-old woman with malignant pheochromocytoma and ectopic ACTH production was reported. Noradrenaline was increased in 24 hour urine and in the blood sample from the left adrenal vein. Hormone assay studies of the tumor tissue and plasma revealed abnormally high levels of ACTH. Formaldehyde fume induced fluorescence method demonstrated biogenic amine in the tumor cytoplasm. Electron microscopic examinations also disclosed numerous neurosecretory granules in the tumor cytoplasm. These findings confirmed that this pleomorphic carcinoma of the left adrenal gland was one of the APUDoma originating from the adrenal medulla, so-called pheochromocytoma.
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PMID:Malignant pheochromocytoma with ACTH production. 609 29

Using a highly specific and sensitive radioimmunoassay for dynorphin(1-13), dynorphin-like immunoreactivity (dynorphin-LI) was detected in two extramedullary pheochromocytomas and a medullary pheochromocytoma. The contents of dynorphin-LI of two extramedullary and a medullary tumors were 9.2, 2.2, and 34.0 pmole/g, respectively. Gel chromatographic studies of tumor extracts on Sephadex G-50 revealed a peak of dynorphin-LI eluting at the same position as dynorphin-LI contaminated in porcine ACTH extract (Sigma) and another portion of dynorphin-LI eluting in a range of molecular weights larger than that of dynorphin-LI in porcine ACTH extract. These results indicate the presence of dynorphin and possibly its precursor peptide(s) in pheochromocytoma.
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PMID:Presence of dynorphin-like immunoreactivity in pheochromocytomas. 611 49

The concentration of epinephrine, norepinephrine, dopamine, met-enkephalin-, ACTH-, calcitonin- and somatostatin-like immunoreactivity (IR) were determined in the extracts of 9 adrenal pheochromocytomas from 7 patients. Six of these patients had Sipple's syndrome. There was a close correlation between the amounts of met-enkephalin-IR and of epinephrine present in the tumor tissue (p less than 0.01). Such a correlation was not found between catecholamines and the other polypeptide hormones investigated. The relevance of the close parallel in the occurrence of met-enkephalin-IR and epinephrine in human adrenal pheochromocytoma tissue is unknown, but it underlines earlier observations in the normal bovine and rat adrenal medulla on a co-storage and co-release of these substances in normal circumstances.
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PMID:A close correlation between the amount of met-enkephalin-immunoreactivity and epinephrine in adrenal pheochromocytoma tissue from patients with Sipple's syndrome. 614 10

We have developed three human cloned cell lines that produce immunoreactive human calcitonin (ihCT) and ACTH (iACTH) as well as exhibit characteristics of cultured neural cells. Clones HMS-41/I, -78/2, and -98/2 were developed from cell lines HeLa AV3, MBA 9812 (bronchogenic carcinoma), and SW 267 (pheochromocytoma), respectively. Karyological analysis of both the parent and the cloned cell lines confirmed the identity of HeLa AV3 and MBA 9812. When grown in serum-free media designed for culturing neural cells, the patterns of production for both ihCT and iACTH varied among the clones. The multiple patterns of hormone production suggest that the mechanisms involved in the biosynthesis, processing, and secretion of these hormones differ among the clones. The clones contain neuron-specific enolase and the putative neurotransmitters beta-alanine and gamma-amino butyric acid, and they respond to cAMP analogs by differentiating, as noted by the extension of neurites (except the HeLa-derived HMS-41/I). The iACTH extracted from cells and synthetic ACTH exhibited equivalent profiles upon isoelectric focusing. The forms of ihCT noted in cell extracts were similar to those observed in extracts of human tumor tissue. Our rabbit antiserum to hCT failed to detect ihCT in those cell extracts prepared for ACTH determination or in extracts of rat pituitaries, but it did detect CT in rat thyroids by both RIA and immunofluorescent procedures. We concluded that our antisera to hCT do not detect the precursor form of ACTH. The availability of these cloned cell lines provides model systems for examining the production of these peptide hormones and the concomitant expression of neural and endocrine characteristics.
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PMID:Characterization of calcitonin- and adrenocorticotropin-producing human cloned cell lines. 627 Jan 87

Most patients with extra-pituitary ACTH-secreting tumors die from carcinoma, but a few can benefit from operation. Of 96 patients with Cushing's syndrome, 11 probably had such tumors. There were three modes of presentation: (1) three had malignant tumors with visceral metastases initially. One (bronchial carcinoid) died without operation. Two with carcinoma (thyroid medullary and islet-cell) underwent adrenalectomy with remission, but died soon. (2) three had apparently benign tumors initially. One (appendicular carcinoid) underwent appendectomy and one (bronchial carcinoid with hilar node metastases) underwent lobectomy. Both had rapid remissions. The third (pheochromocytoma) died after resection of the tumor. (3) five patients had no obvious tumors and underwent adrenalectomy with remission. In one a benign bronchial carcinoid was removed later. Four others remain well, but without localizing signs of tumor. The main biochemical features in all were hypokalemic alkalosis and very high urinary excretion of free cortisol. Seven of the eight patients without visceral metastases are in remission from one to 15 years after operation.
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PMID:Surgical management of the ectopic ACTH syndrome. 628 49

ACTH-stimulation adrenal imaging (ACTH-I) was performed in 14 patients after baseline imaging (B-I) was performed. In six patients with no adrenal diseases in whom the findings of B-I were equivocal, morphologic normality of the adrenals was confirmed by ACTH-I because of increased adrenal uptake of radioiodocholesterol. In three patients with cortisol-producing tumors, visualization of contralateral glands by ACTH-I provided indirect evidence for autonomous cortisol secretion of the tumors. In three patients with pheochromocytoma or cyst, ACTH-I increased adjacent cortical radioactivity to more clearly delineate the lesions. In two patients with primary adrenocortical insufficiency, exogenous ACTH had no effect on adrenal uptake of the tracer. Correlation was observed between response of the adrenal net counts and urinary excretion of 17-OHCS and 17-KS. ACTH-I is useful when B-I does not provide sufficient diagnostic information or further information is needed due to low or absent radioiodocholesterol uptake by the adrenal gland(s).
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PMID:Application of ACTH stimulation to adrenal imaging with radioiodocholesterol. 630 67

Immunoreactive corticotropin-releasing factor (I-CRF) and ACTH (I-ACTH) were examined using RIA, immunoaffinity chromatography, and gel filtration chromatography in human hypothalamus, adrenal (cortex and medulla), lung cancer, and pheochromocytoma. I-CRF and I-ACTH were present in these tissues. Gel filtration of I-ACTH in the adrenal, pheochromocytoma, and lung cancer showed the presence of larger amounts of I-ACTH with large molecular weight forms in contrast to the hypothalamus. Gel filtration of I-CRF in these tissues showed the main peak eluted at the position of synthetic rat CRF. High performance liquid chromatography of this main peak showed two components which eluted in the positions of synthetic rat CRF and oxidized CRF. These elution positions were the same in all tissues and identical with those in the hypothalamus. These results suggest the presence of I-ACTH and I-CRF in these tissues and that CRF outside the brain is identical to hypothalamic CRF.
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PMID:Immunoreactive corticotropin and corticotropin-releasing factor in human hypothalamus, adrenal, lung cancer, and pheochromocytoma. 632 18

A 47-year old woman is presented who had a left adrenal pheochromocytoma with manifestations also indicative of ectopic ACTH syndrome. The excised adrenal gland showed both an adrenal medullary tumor and adrenal cortical hyperplasia. Immunostaining also showed the presence in the tumor of ACTH and beta endorphins. The immediate post-adrenalectomy decline of catecholamines, ACTH, cortisol and beta endorphins indicate that the adrenal itself was the source of these hormones. This patient, the thirteenth now reported in the literature, represents one form of ectopic ACTH syndrome in which the lesion is benign, and in which a successful outcome can be anticipated.
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PMID:Report of a case of pheochromocytoma producing immunoreactive ACTH and beta-endorphin. 632 97


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