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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Case reports of neuroblastoma revealed that some individuals are genetically predisposed and that this genetic predisposition may have other consequences. According to a mutation model, two classes of individuals could acquire neuroblastoma. One (prezygotic) was a rare class that carried a dominant gene imparting high risk of the tumor. The other (postzygotic) comprised all other individuals, each at low risk. The model related tumor incidence to germinal and somatic mutation rates and thereby carried implications for environmental modification of tumorigenesis and demographic variation in incidence. Case reports also revealed associations of neuroblastoma with congenital defects and a susceptibility to second tumors. Analogy with retinoblastoma and Wilms' tumor of the kidney suggested that these associations could result from action of a neuroblastoma gene or from chromosomal aberration. One known dominantly inherited condition, von Recklinghausen's disease, could dispose to neuroblastoma and create some associations. According to the two-mutation model, neuroblastoma may have been a single recessive gene disorder at the level of the cell. The phenomena of aganglionosis, neuroblastoma in situ, maturation of neuroblastoma to ganglioneuroma, and spontaneous regression suggested that such a neuroblastoma gene interfered with normal developmental processes. The specificities of this gene and of those for von Recklinghausen's disease and pheochromocytoma suggested that the functiof a membrane macromolecule.
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PMID:Developmental genetics of neuroblastoma. 18 2

A case of bilateral pheochromocytoma with an associated pancreatic islet cell tumor is reported. Prominent calcification was noted in the left adrenal tumor.
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PMID:Pheochromocytoma with prominent calcification and associated pancreatic islet cell tumor. 19 50

Two unusual cases of the watery diarrhea syndrome are presented. In one patient an adrenal medullary tumor, a pheochromocytoma that produced vasoactive intestinal polypeptide (VIP) was excised with total relief of symptoms. The second patient a 65-year-old man with abrupt onset of massive watery diarrhea that led to acidosis and coma was symptomatically controlled for one year on 10 mg/day of prednisone. Elevated levels of VIP returned to normal after prednisone therapy was started. A benign islet cell tumor not localized by angiography was removed by distal pancreatic resection. Tissue levels of VIP were markedly elevated. VIP is a humoral mediator of the water diarrhea syndrome. Both benign and malignant pancreatic and extrapancreatic tumors may cause the watery diarrhea syndrome. Steroids may cause symptomatic relief of the diarrhea by lowering peptide levels to normal. The term watery diarrhea syndrome may be more accurate than the pancreatic cholera syndrome.
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PMID:Watery diarrhea syndrome. Two unusual cases and further evidence that VIP is a humoral mediator. 20 79

A 14-year-old Japanese male with a previously undescribed combination of bilateral pheochromocytoma and an islet cell tumor of the pancreas is presented. The combination of endocrine neoplasms in this patient overlaps multiple endocrine neoplasia (MEN) Type 1 and Type 2. A total of 14 reported cases of MEN overlapping Type 1 and Type 2 is reviewed. Of the 14, 7 patients with acromegaly developed a paraganglioma(s), 2 patients with Sipple syndrome had a pituitary adenoma, and in the other 5 patients, an intestinal carcinoid or a pancreatic islet-cell tumor occurred in association with either a thyroid medullary carcinoma or a paraganglioma(s). We believe that the occurrence of MEN overlapping Type 1 and Type 2 is more than a fortuitous association, and can be explained on the basis of the neuroectodermal origin.
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PMID:Coexistence of bilateral pheochromocytoma and pancreatic islet cell tumor: report of a case and review of the literature. 21 1

A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure.
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PMID:Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature. 22 80

The authors report 4 cases of pheochromocytoma and analyse the interest of the different x ray examinations. The authors show the interest of angiography, particularly selective angiography, for small tumors, ectopic and multiple abdominal localisations, and metastases. The benign or malignant nature of the tumor, in the absence of metastases, is difficult to assess by x ray examinations alone.
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PMID:[Value of radiological examination in patients with pheochromocytoma. Results in four cases (author's transl)]. 23 32

A patient found to have a pheochromocytoma in the 16th wk of pregnancy was successfully managed by medical treatment with phenoxybenzamine hydrochloride and propranolol. In the 38th wk of pregnancy she was electively delivered by cesarean section, and the tumor was simultaneously removed.
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PMID:Successful control of pheochromocytoma in pregnancy. Case report. 26 93

The cytologic and histochemical characteristics of the tumor cells from medullary thyroid carcinoma and pheochromocytoma are described. In cell samples obtained from medullary thyroid carcinomas, amyloid deposits were detected both intracellularly and extracellularly. Medullary thyroid carcinoma cells, as well as pheochromocytoma cells, showed positive for both argentaffin and argyrophil reactions. The diagnostic significance of intracellular amyloid deposits and of positive silver and chromaffin reactions of the intracytoplasmic granules may be stressed in establishing the cytologic diagnosis of medullary thyroid carcinoma and/or pheochromocytoma.
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PMID:Cytologic appearances of pheochromocytoma and medullary thyroid carcinoma occurring within a family. 28 38

In six patients with adrenal pheochromocytoma the tumors were localized by ultrasonography, phlebography of the adrenal glands and by estimation of plasma catecholamines selectively obtained from the vena cava and the adrenal gland veins. All tumors were localized by selective catecholamine estimation, five by ultrasonography, and four by phlebography. The smallest pheochromocytoma of 1.5 g weight was only localized by selective catecholamine estimation but not by ultrasonography or phlebography. This tumor, however, had been visualized by computed tomography. To avoid diagnostic errors by selective catecholamine estimation, it is important to withdraw blood from the adrenal gland veins prior to the injection of any radiographic contrast media, since this may result in an extremely enhanced secretion of catecholamines from the adrenal medulla.
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PMID:[Modern methods in localization of pheochromocytomas (author's transl)]. 31 80

Adrenal cysts are rare clinical and pathologic entities. The vast majority are minute in size, unilateral, and found in females at autopsy. They come to the physician's attention when they produce symptoms or are identified on x-ray studies. When symptomatic, they generally produce lumbar discomfort and gastrointestinal symptoms, and occasionally are palpable by abdominal examination. There is no significant agreement as to cause and pathogenesis of the disease. The most common histologic types are lymphangiomatous endothelial cysts, secondary to lymphangiectasis, and the fibrous wall or hemorrhagic pseudocysts. In many patients preoperative diagnosis is now possible with high quality nephrotomography, ultrasonography, and adrenal arteriography. The presence of a suprarenal mass with peripheral or laminar calcification strongly suggests the presence of an adrenal pseudocyst. Selective biochemical studies are mandatory to rule out the presence of an occult cystic pheochromocytoma, and adrenal cortical tumor. Adrenal cysts must be differentiated from all space-occupying lesions of the upper abdomen. Surgical exploration is recommended in almost all patients for accurate diagnosis and to rule out malignant disease or occult pheochromocytoma. The choice of the surgical approach should be planned to provide for safe and adequate exposure, depending on the size and location of the lesion. Careful dissection with preservation of the adjacent kidney, liver, and pancreas should be performed.
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PMID:Functioning and nonfunctioning cysts of the adrenal cortex and medulla. 33 76


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