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

Multiple endocrine neoplasia, type 2b, is a disorder of unknown etiology with major involvement of the thyroid and adrenal glands, the autonomic nervous system, and connective tissue. It is transmissible with an autosomal dominant pattern of inheritance, but since most cases are not familial, they presumably represent mutations. The thyroid gland exhibits bilateral medullary carcinoma, which is a metastasizing lethal neoplasm in the syndrome requiring total thyroidectomy once abnormal basal or stimulated concentrations of plasma immunoreactive calcitonin have been demonstrated. The adrenal medullary tumors--pheochromocytomas--although rarely malignant, are potentially lethal because of their cardiovascular effects. Since the adrenal involvement is usually bilateral, total bilateral adrenalectomy with excision of any extraadrenal paraganglioma is the surgical treatment. Parathyroid hyperplasia occurs rarely in the syndrome. Treatment of it should be conservative, that is, limited to excision of enlarged parathyroid glands. Major portions of the autonomic nervous system, both sympathetic and parasympathetic, nerves and ganglia, exhibit hypertrophy, hyperplasia, and disorder of structure--a group of changes designated ganglioneuromatosis. This may be largely responsible for the striking eye and oral findings--the hallmarks of the syndrome--and also for some of the serious symptoms and complications of the syndrome, particularly those referable to the alimentary tract. Ganglioneuromatosis is also found in the salivary glands, pancreas, gallbladder, upper respiratory tract, and urinary bladder. The connective tissue abnormality is manifested by increased growth of long bones, ribs, and skull, resulting in a marfanoid habitus, and also by skeletal and joint abnormalities together with increased laxity of ligaments. Ninety cases of MEN 2b have been reported, and although follow-up information is incomplete, 27 patients (30 percent) are known to be dead because of the syndrome. The causes of death have been medullary thyroid carcinoma (15 deaths), pheochromocytoma (10 deaths), and alimentary tract complications (2 deaths). An additional 21 patients (22 percent) are known to have metastatic MTC. We are aware of only 2 patients who, 5 years after thyroidectomy, have apparently been cured of MTC, but both are still at risk for adrenal medullary disease. MEN 2b is, therefore, a very serious disorder that requires urgent treatment of the endocrine tumors. Fortunately, the majority of patients with the syndrome are easily recognized because of an abnormal phenotype typified by thick, bumpy lips and a marfanoid habitus. Since these findings signal high risk for the potentially lethal endocrine neoplasms, patients having the characteristic appearance need evaluation of thyroidal C-cell and adrenal medullary function.
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PMID:Multiple endocrine neoplasia, type 2b. 36 72

Pheochromocytomas are usually hypervascular tumors exhibiting dense stain during angiography. The cystic nature of the neoplasm or tumor necrosis can result in failure of opacification of the mass or a central lucent area. Four cases of pheochromocytoma are described where a central avascular zone was surrounded by a dense rim of contrast, giving a "ring sign." Since this appearance is best seen in the capillary and venous phases, delayed films are essential.
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PMID:The "ring sign" of necrotic pheochromocytoma. 42 8

The diagnosis "pheochromocytoma" is established by the evaluation of catecholamins and their metabolites in urine. Therapy consists in extirpation of the hormone producing tumor. Aim of he X-ray examination is the localization of the tumor before operation. 80% of the pheochromocytomata are located in the suprarenal glands. As there is the possibility of extraadrenal localization (20%) and of multicentric manifestation (11%) the X-ray examination must not be restricted to the suprarenal glands. More than 95% of all pheochromocytomata are well vascularized, therefore abdominal aortography is the best method to search for and localize these tumors. This invasive method necessitates close teamwork of the radiologist and a physician with great experience in this field. This is necessary for the preparation of the patient for aortography and also for the treatment of possible complications during aortography, which are specific for pheochromocytoma and which can be fatal.
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PMID:[Radiodiagnosis in pheochromocytoma]. 43 55

Localization of adrenal lesions in various adrenal disorders can be difficult. An attempt to identify the adrenal tumors in ten patients with pheochromocytoma, Cushing's syndrome, or primary aldosteronism was made using computerized tomographic (CT) scans. The adrenal tumor was visualized in eight patients. The CT scan appears to be a promising noninvasive technique for localization of adrenal tumors.
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PMID:Detection of adrenal tumors by computerized tomographic scan in endocrine hypertension. 44 54

A 24-year-old man with diagnosed pheochromocytoma of the organ of Zukerkandl, was successfully treated with propranolol hydrochloride to lower his blood pressure. In view of the positive response to propranolol, the patient was expected to have an epinephrine-secreting tumor. However, measurement of catecholamines showed that preoperative peripheral blood, vena caval samples from the tumor, intraoperative peripheral samples, and the tumor itself contained almost exclusively norephinephrine. A response of blood pressure to propranolol in a hypertensive patient does not rule out the possibility of a pheochromocytoma, even one with the usual norephinephrine predominance.
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PMID:Norepinephrine-secreting tumor of the organ of Zuckerkandl. Response to propranolol alone. 44 87

Long-term epidemiological and laboratory studies were carried out in a kindred with familial pheochromocytoma associated with von Hippel-Lindau disease. Thirteen members were affected by the syndrome and the trait appears to be transmitted in an autosomal dominant fashion. Of 13 patients, 7 had pheochromocytoma alone. Of the remaining six patients, one had pheochromocytoma combined with von Hippel-Lindau disease, four had pheochromocytoma with retinal disease only, and a single patient had a retinal lesion without pheochromocytoma. In four patients, pheochromocytoma antedated the development of retinal lesions. Ten members also had mild hypercalcemia without accompanying elevations of PTH in the 4 patients in whom this was determined. In all, hypercalcemia was corrected with removal of tumors, and no patient had a return of hypercalcemia in the absence of recurrent increases in urinary catecholamines. The clinical presentations in 12 patients varied markedly, as did their urinary excretion rates of norepinephrine, epinephrine and their metabolites. However, an analysis of the data revealed significant correlations not previously described between the urinary excretion of free catecholamines (norepinephrine plus epinephrine), blood pressure, the free catecholamine content of the tumor and the age of the patient. Urinary excretion of free norepinephrine plus epinephrine appear to be decreased with advancing age (p less than 0.001). Both systolic and diastolic blood pressures and the age of the patient were inversely correlated (p less than 0.01). A significant inverse relationship between the tumor content of free catecholamines and the age of the patients was, although to a lesser degree, also present (p less than 0.05). As a whole, the size of the tumors and their norepinephrine content were not correlated. We present a concept that, in familial pheochromocytoma, the metabolism of catecholamines is altered by the process of aging, and that this change modifies the clinical presentations of the disease.
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PMID:Familial pheochromocytoma, hypercalcemia, and von Hippel-Lindau disease. A ten year study of a large family. 44 57

The outer medullary (juxtacortical) zone of a normal equine adrenal gland had columnar chromaffin-positive cells arranged with their long axes perpendicular to fine vascular channels. The deeper medullary regions were composed of smaller irregularly round to polygonal chromaffin positive cells in small packets. Both cell types contained two types of membrane-bound cytoplasmic secretory granules. Osmiophilic granules with a homogeneous core, crenated membrane and narrow submembranous halo predominated in the columnar juxtacortical cells. The rounder, central medullary cells contained predominantly electron dense granules with a wide irregular electron lucent space between an eccentric core and the granule membrane. In contrast, irrespective of cell type or zone, cells from a pheochromocytoma contained only one type of granule similar to that described for the juxtacortical region of the normal equine adrenal medulla. The tumor cells could be classified into three subtypes based on density of granule packing but the granules were morphologically similar in all tumor cells.
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PMID:A light and electron microscopic study of a normal adrenal medulla and a pheochromocytoma from a horse. 45 13

The authors have studied the prognostic value of such convincing morphological signs of tumor malignancy as cellular and nuclear polymorphism, the mitotic activity, the invasion of tumor cells into the vascular lumen, the presence of metastases in 28 operated patients with a hormone-active tumor of chromaffin tissue. The results of late-term observations within the period from 2 to 17 years indicated that all the patients subjected to radical surgery are alive. Detecting in a histological assay of pheochromocytoma of the aforementioned signs of malignancy except metastases is not an obligatory prognostically unfavourable factor. Such tumor cannot be called pheochromoblastoma, but it should be defined as pheochromocytoma with the morphological signs of malignancy.
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PMID:[Prognostic significance of the morphological signs of the malignancy of a pheochromocytoma]. 46 72

A 17-year-old woman manifested fever, abdominal pain, headache, and hypertension caused by a solitary, benign pheochromocytoma. She also had hypercalcemia and elevated plasma immunoreactive calcitonin levels. After removal of the pheochromocytoma, calcium and calcitonin levels returned to normal. Studies of peripheral and tumor venous blood showed no excess or ectopic parathyroid hormone secretion, but the tumor contained and secreted calcitonin. Sporadic pheochromocytoma may secrete calcitonin and cause hypercalcemia by non-parathyroid hormone-mediated mechanisms. The potential is clearly present for confusion with multiple endocrine neoplasia, type 2 (medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism).
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PMID:Pheochromocytoma associated with hypercalcemia and ectopic secretion of calcitonin. 46 64

A 26-year-old woman had a neuroblastoma during infancy; an extra-adrenal pheochromocytoma at age 16 years, with hepatic recurrences during the next ten years; and multifocal renal cell carcinoma. Neuroblastoma and pheochromocytoma, despite their common embryologic origin, to our knowledge have not been previously reported as separate tumors in the same patient. Although many attributes of the patient's tumors suggest a hereditary disorder, thorough investigation disclosed no evidence of heritable conditions associated with any of these tumors in the patient or her family members. Long-term observation of persons surviving after treatment of neuroblastoma will be necessary to determine whether this case represents a previously unidentified tumor predisposition or a sporadic occurrence.
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PMID:Neuroblastoma, pheochromocytoma, and renal cell carcinoma. Occurrence in a single patient. 49 Aug 9


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