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)

A collection of 13 tumors causing an ectopic Cushing's syndrome was composed of four bronchial carcinoids, one small cell carcinoma of the bronchus, one thymic carcinoid, two islet cell tumors of the pancreas, one pheochromocytoma, two medullary carcinomas of the thyroid, one prostatic cancer and one intrasellar choristoma. By immunohistochemistry, ACTH in combination with CRH was found in one bronchial carcinoid and in one islet cell carcinoma. ACTH but not CRH was demonstrable in one bronchial carcinoid, in both medullary carcinomas of the thyroid, in the thymic carcinoid and in the pheochromocytoma. CRH without ACTH was present in the small cell carcinoma of the bronchus, one bronchial carcinoid, the prostatic cancer and the choristoma of the sellar region. Neither ACTH nor CRH could be found in one islet cell carcinoma. In the pituitary (n = 7) Crooke's cells were found except in one case with islet cell carcinoma which was treated with adrenostatic drugs. The pituitary besides the intrasellar choristoma harbored an ACTH cell adenoma. The pathophysiological correlations are discussed.
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PMID:[Ectopic ACTH- or CRH-secreting tumors in Cushing's syndrome]. 839 19

Endocrine crises can occur in diabetes mellitus, in pituitary failure when there is a lack of ACTH, TSH or ADH secretion, in severe hyper- or hypothyroidism (thyroid storm and myxedema coma), severe hyper- or hypoparathyroidism (parathyroid crisis and tetany), in adrenal failure and in patients with pheochromocytoma or carcinoid tumors. Cushing's syndrome can be associated with psychotic crises. This review describes the most important clinical features and the basic diagnostic and therapeutic aspects of the non diabetic endocrine crises.
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PMID:[Endocrine crises]. 848 76

This paper reports the experience of five Belgian surgical teams with 18 videoendoscopic adrenalectomies performed on 16 patients between October 1993 and May 1995. The adrenal gland diseases were pheochromocytoma (4 patients), primary hyperaldosteronism (2 cases), Cushing's adenoma (2 cases), Cushing's disease (1 case), nonfunctional adenoma (3 cases), single metastasis from adenocarcinoma (2 cases), functional adenoma with dehydro-epiandrostenedione (DHEAS) and cortisol hypersecretion (1 case), ACTH secreting metastases from a thymoma (1 case) Two patients underwent bilateral adrenalectomies. Eleven left and three right adrenal glands were removed in 14 other patients. The eight women and eight men range in age from 17 to 72 years (median 47). Six patients demonstrated a body mass index greater than 30. Median tumor size was 3 cm (range 1.3 - 5). Laparoscopic adrenalectomy was successful in 14 patients (87%). The median duration of the procedure was 132 minutes (range 59-360). The median postoperative stay was 6 days (range 2-13). No patient required blood transfusion. We conclude that the videoscopic approach can safely be used for surgical removal of adrenal lesions. However this approach should be performed by surgeons well versed in the techniques of open adrenalectomy for endocrine disorders, but also well trained in videoendoscopic surgery.
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PMID:Videoendoscopic adrenalectomy: multicentric study from the Belgian Group for Endoscopic Surgery (BGES). 880 96

A young female adult with Cushing's syndrome arising from ectopic production of corticotropin (ACTH) from an adrenal pheochromocytoma showed spontaneous clinical and biochemical remission of hypercortisolism after a brief period of ketoconazole administration. Despite continued remission of the hypercortisolism over the next 18 months, there was progressive catecholamine hypersecretion with significant morbidity as a result of the pheochromocytoma. Surgical resection of the left adrenal gland revealed a pheochromocytoma showing focal cytoplasmic immunostaining for ACTH and marked diffuse compact cell hyperplasia in the adrenocortical tissue. To our knowledge this is the first reported case of spontaneous clinical and biochemical remission of ectopic ACTH production from a pheochromocytoma. The pathogenesis of the remission remains unknown.
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PMID:Spontaneous remission of ectopic Cushing's syndrome due to pheochromocytoma: a case report. 892 26

The subcellular localization and functionality of transfected yeast aspartic protease 3 (YAP3p) in a mammalian cell line were investigated. The complementary DNAs encoding the prohormone-processing enzyme (YAP3p) and a prohormone, bovine POMC, were cotransfected into PC12 (rat pheochromocytoma) cells. Immunocytochemical analysis of the cells using a YAP3p antibody showed a perinuclear punctate distribution of YAP3p in the cell body as well as immunostaining in the tips of the neurites. This pattern of immunostaining indicates localization of YAP3p in secretory granules. Analysis of the processing of POMC showed that in cells transfected with the POMC complementary DNA alone, only POMC was found, indicating a lack of processing of the prohormone. However, in cells coexpressing YAP3p, the POMC was completely processed to yield ACTH-(1-39) and ACTH-(1-14), consistent with the specificity of YAP3p found in vitro. Pulse-chase studies showed that POMC was processed after 20 min of chase, suggesting that processing occurred in the late Golgi network and continued in the secretory granules. Western blot analysis determined that YAP3p was secreted from the cells in a regulated manner. This study provides the first demonstration that a yeast prohormone-processing enzyme (YAP3p) of the aspartic protease class can be sorted correctly to secretory granules and activated to process a prohormone (POMC) in a highly efficient manner in mammalian cells.
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PMID:Yeast aspartic protease 3 is sorted to secretory granules and activated to process proopiomelanocortin in PC12 cells. 894 Mar 69

The discovery of an asymptomatic adrenal mass (incidentaloma) during the investigation of an unrelated condition is relatively common. In this study, we report the clinical, radiologic, and endocrine evaluation of 38 patients (22 women and 16 men aged 24 to 84 years) with adrenal incidentaloma (size, 1 to 12 cm). The patients underwent basal and dynamic evaluation of the hypothalamic-pituitary-adrenal (HPA) axis, renin-angiotensin-aldosterone system, and adrenomedullary function. Moreover, computed tomograpy (CT) scan and 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol(NP-59) and/or 131I-metaiodobenzylguanidine (MIBG) scintigraphy were performed. The endocrine evaluation indicated two cases of pheochromocytoma and four cases of preclinical Cushing's syndrome, three of which underwent surgery with histologic diagnosis of two adrenocortical adenomas and one carcinoma. Low levels of serum dehydroepiandrosterone sulfate (DHEA-S), associated with a markedly increased 17-hydroxyprogesterone (17-OHP) response to a corticotropin (ACTH) test, were found in patients with incidentaloma. On the basis of endocrine and morphologic data, 13 patients underwent surgical treatment: five adrenocortical adenomas (two functioning), two pheochromocytomas, two ganglioneuromas, one cortisol-secreting adrenal carcinoma, one lymphangiomatous cyst, one myelolipoma, and one hemorrhage were found. Careful diagnostic assessment of incidentally discovered adrenal masses must be performed to exclude the presence of malignant and/or functioning lesions and to verify the possibility that patients with incidentaloma have a genetic or acquired deficit of adrenal steroidogenic activity.
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PMID:Evaluation of hormonal function in a series of incidentally discovered adrenal masses. 900 78

Adrenal masses are more and more frequently detected by adrenal ultrasound, computed tomography or nuclear magnetic resonance carried out for a reason other than the suspicion of adrenal disease (incidentalomas). The findings of an incidentaloma still leaves many diagnostic and therapeutic questions open. We report the results of a multicentric retrospective evaluation of patients with adrenal incidentalomas, performed by a Study Group of the Italian Society of Endocrinology. According to the definition of incidentaloma, exclusion criteria a priori were: severe or paroxysmal hypertension, frank hypokalemia and clinical signs of hypercortisolism or hyperandrogenism. 29 centers participated in the study and the data obtained by questionnaire were collected in 2 centers for final elaboration. Center 1 carried out the epidemiological and clinical evaluation. Basal and dynamic hormonal evaluation of 786 among the 1013 cases recruited were performed in our center (center 2). Functional studies included: diurnal rhythm of cortisol, urinary free cortisol (UFC), ACTH, DHEAS, 17-OH progesterone, testosterone, androstenedione, supine and upright plasma renin activity (PRA) and aldosterone, urinary aldosterone, urinary catecholamines and VMA. The hormonal dynamic evaluation included the overnight dexamethasone suppression test (1 mg), CRH test and ACTH test. In our study, 89% (702 patients) of adrenal incidentalomas were non-hypersecretory masses; 6.2% (49 patients) showed a preclinical Cushing's syndrome (PCS) (at least two altered parameters of pituitary-adrenal axis); 3.4% (27 patients) were pheochromocytomas; 0.89% (7 patients) were aldosteronomas. One tumor was a masculinizing adrenocortical carcinoma. Two hundred sixty patients underwent surgical exploration and the histological diagnosis showed: 138 adenomas (53%), 32 carcinomas (12%), 26 pheochromocytomas (10%). 16 myelolipomas (8%), 13 cystic lesions (5.5%), 7 tumors of neuronal lineage (3%). 12 metastases (4%), 13 others (5%). The 138 patients with adenomas had the following hormonal diagnosis: 103 nonfunctional adenomas (74%), 31 PCS (23%) and 4 cases of hyperaldosteronism (3%). In the patients with PCS an abnormal dexamethasone suppression test was found in 86% of cases (37/41 patients). Values for ACTH were low in 78% (32/41 patients). UFC was elevated in 64% of patients, the diurnal rhythm of cortisol evaluated in 14 patients was absent in 7. Only in 50% of cases DHEAS values (12/24 patients) were decreased, whereas they were normal in the other 50%. Interestingly, 8 patients with normal DHEAS and normal UFC showed nonsuppressible cortisol by dexamethasone test (1 mg). Blunted ACTH response to CRH was detected in 9 of 14 patients (64%). Thus our data suggest that the best parameter for evaluating subclinical hypercortisolism seems to be the overnight dexamethasone suppression test. In 27 patients with pheochromocytoma 24-hour urinary catecholamine and VMA levels were elevated in 86 and 46% of cases respectively. In 7 patients with hyperaldosteronism upright PRA was suppressed in 100% of cases and aldosterone plasma levels were elevated in 6 patients (86%); serum potassium level was slightly decreased in 60% of cases. In 86 of 138 histologically proven adenomas, DHEAS levels were: normal in 59% of patients, decreased in 36% and elevated in 4.6%, whereas in 22 of 32 cortical carcinomas evaluated. DHEAS levels were normal in 63% of cases, decreased in 18% and elevated 18%. Post-ACTH 17-OH progesterone levels were elevated in 52% (62/118 patients) of non-functioning adenomas and in 2 of 4 carcinomas. Not enough data are yet available postoperatively. In summary, endocrine evaluation can lead to the identification of a nonnegligible number of cases of clinically unsuspected pheochromocytomas and subtle hypercortisolism (about 3.4 and 6.2%, respectively of all adrenal incidentalomas), while cases of primary subclinical aldosteronism are rarely found. (ABSTRACT TRUNCATED)
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PMID:Adrenal incidentaloma: an overview of hormonal data from the National Italian Study Group. 916 66

KAT45 cells were derived from a human pheochromocytoma, which also caused ectopic Cushing's syndrome, and developed into a cell line spontaneously after the continuous primary culture of the tumor cells. These human pheochromocytoma cells were compared with the extensively characterized PC12 rat pheochromocytoma cell line. KAT45 cells resembled PC12 cells in morphology, proliferation rate, response to cholinergic stimuli, and the development of dendrite-like projections after exposure to nerve growth factor. They produced norepinephrine and epinephrine in a ratio of 50:1, as opposed to production of dopamine by PC12 cells, in amounts 1 order of magnitude higher compared with PC12. Because of the ectopic Cushing's syndrome in our patient, her normal ACTH level, and the knowledge that PC12 cells and even normal rat chromaffin cells appear to produce CRH, we examined whether KAT45 cells also produced this neuropeptide. Indeed, KAT45 cells released authentic CRH and contained an apparently intact CRH transcript. Nicotine and KCl depolarization stimulated the secretion of CRH, whereas interleukin-1beta, glucocorticoids, and nerve growth factor stimulated its synthesis. In addition to the potential systemic effects of CRH, which in our patient produced ectopic Cushing's syndrome, CRH can exert paracrine effects within normal or tumoral adrenals. We used KAT45 cells as a model for the study of the local role of CRH. CRH affected several parameters of KAT45 cell metabolism, including their proliferation rate, synthesis of catecholamines, and production of POMC-derived peptides. KAT45 cells, in addition to the data they provided regarding the in vitro profile of a human CRH-producing pheochromocytoma, may prove to be a valuable auxiliary to the PC12 cell line.
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PMID:KAT45, a noradrenergic human pheochromocytoma cell line producing corticotropin-releasing hormone. 944 45

In a 10-year-old ovariohysterectomized standard Schnauzer, the finding of dexamethasone-resistant hypersecretion of cortisol, the results of computed tomography, and elevated plasma concentrations of ACTH suggested the presence of both adrenocortical tumour and pituitary-dependent hyperadrenocorticism. The dog made an uneventful recovery after bilateral adrenalectomy and remained in good health for 31/2 years with substitution for the induced hypoadrenocorticism. Then the enlarged pituitary caused neurological signs and eventually euthanasia was performed. The surgically excised right adrenal contained a well-circumscribed tumour of differentiated adrenocortical tissue and in the left adrenal there were two adrenocortical tumours and a pheochromocytoma. The unaffected parts of the adrenal cortices were well developed and without regressive transformation. At necropsy there were no metastatic lesions. The cells of the pituitary tumour were immunopositive for ACTH and had characteristics of malignancy. The present combination of corticotrophic tumour, adrenocortical tumours, and pheochromocytoma may be called 'multiple endocrine neoplasia' (MEN), but does not correspond to the inherited combinations of diseases known in humans as the MEN-1 and the MEN-2 syndromes. It is suggested that the co-existence of hyperadrenocorticism and pheochromocytoma may be related to the vascular supply of the adrenals. Some chromaffin cells of the adrenal medulla are directly exposed to cortical venous blood, and intra-adrenal cortisol is known to stimulate catecholamine synthesis and may promote adrenal medullary hyperplasia or neoplasia.
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PMID:Multiple endocrine neoplasias in a dog: corticotrophic tumour, bilateral adrenocortical tumours, and pheochromocytoma. 956 61

Normal rodent adrenal chromaffin cells and the PC12 rat pheochromocytoma cell line produce IL-1 cytokines. The role, if any, of these cytokines is currently unknown. In PC12 cells, they induce the expression of the L-AA decarboxylase mRNA, a major step in the biosynthesis of catecholamines. Very little if any of these cytokines are detectable in normal human adrenal medulla, being confined mainly in the 17 alpha-hydroxylase-positive steroid cells of the zona reticularis. The aim of the present work was to find out if human pheochromocytomas produce IL-1 cytokines, in vitro, and to examine what local role they may exert. As a model, we have used the new KAT45 cell line, which emerged spontaneously from a primary human pheochromocytoma cell culture. We have found that the KAT45 cells secrete IL-1 beta at 47.8 +/- 9 pg/mg total cellular protein (n = 7, at 24 hours). IL-1 beta increased the concentration of norepinephrine in the KAT45 culture medium from 24.2 +/- 3.5 micrograms/mg protein (n = 6 controls, at 24 hours), to 33.2 +/- 3.5 (IL-1 beta 10 mg/ml) or to 42.9 +/- 8 (IL-1 beta 30 mg/ml). This effect was blocked by IL-1ra. The KAT45 cells also produce CRH and ACTH. IL-1 beta stimulated the secretion of CRH from 19.2 +/- 4 pg/mg protein (n = 5 at 36 hours) to 38.7 +/- 4, an effect blocked by IL-1ra in excess. IL-1 beta had no effect on ACTH secretion.
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PMID:KAT45 human pheochromocytoma cell line. A new model for the in vitro study of neuro-immuno-hormonal interactions. 962 69


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