Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinically significant adrenal hemorrhage due to adrenal metastases has rarely been reported. We describe three cases of this unusual entity, two from lung carcinoma and one from sarcoma. Adrenal hemorrhage was bilateral in two patients, and was the presenting manifestation of malignancy in two. A computed tomography diagnosis of hemorrhage within bilateral adrenal metastases was made when adrenal masses rapidly enlarged in one case, and when previously resolving hematomas showed enlargement in a second case. The third case was believed to represent a pheochromocytoma preoperatively. We conclude that significant adrenal hemorrhage can result from adrenal metastases and can be the presenting manifestation of metastatic tumor. In the patient without discernible risk factors for adrenal hemorrhage, underlying malignancy should be considered as a possible cause, even if the hemorrhage is bilateral.
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PMID:Clinically significant adrenal hemorrhage secondary to metastases. Computed tomography observations. 281 83

Besides the juxtaglomerular cell tumors, tumors may be responsible for a primary hyperreninism syndrome. Strict criteria allow to assert that the tumor cells themselves are involved in ectopic renin secretion. They are as follows:--measurement of the renin in the blood and in tumoral tissue extracts with assessment of active anf inactive renin,--absence of any other cause of hyperreninism,--regression of the hyperreninism when the tumor is removed, and possibly recurrence when metastases appear,--demonstration of renin antigen in tumor cells by immuno-histochemistry and more recently detection of renin messenger RNA using in situ hybridization with human renin probe. About 40 cases of these tumors have been described. They are mainly renal tumors: nephroblastomas (29 cases), adenocarcinomas (7 cases) and other rare tumors. Among extrarenal tumors, it has been observed epithelial tumors (broncho-pulmonary cancers, ovarian, fallopian and pituitary tumors), soft tissue tumors (alveolar sarcomas. epithelioid sarcoma, hemangiopericytoma, leiomyosarcoma). It has not been demonstrated that tumor cells from pheochromocytoma could be themselves involved in renin production.
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PMID:[Tumor syndromes with inappropriate renin secretion. Diagnostic criteria and review of published cases]. 284 Sep 23

Ultrasound screening in 9 and 21 members, respectively, of two families affected by familial C-cell carcinoma, as well as sonographic findings in 6 patients with sporadic medullary carcinoma of the thyroid (MCT), are reported. Unilateral and bilateral tumor nodules were identified by sonography in 12 of a total of 13 patients with MCT (n = 9) or local tumor recurrence following thyroidectomy (n = 4); one carcinoma, 3 X 6 mm in diameter, was missed. Nine of the 12 (75%) were clinically occult, nonpalpable C-cell carcinomas. The smallest occult MCT correctly diagnosed by ultrasound had a diameter of 4 mm. A positive correlation was found between the sonographically determined tumor mass (number/size of nodules/metastases) and the plasma calcitonin level. Pheochromocytomas were diagnosed by ultrasound as part of a multiple endocrine neoplasia (MEN IIa) in two patients with MCT. The echomorphologic findings of the intrathyroid C-cell carcinomas and their metastases display characteristic but nonspecific features. Sonographic findings on these tumors should therefore not be interpreted without consideration of plasma calcitonin assays.
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PMID:Ultrasound diagnosis of C-cell carcinoma of the thyroid. 285 98

The efficacy of the newly developed pheochromocytoma-seeking radiopharmaceutical, [131I]MIBG, was examined in the first 400 patients (441 studies) investigated for suspected pheochromocytoma at our institution. The results of [131I]MIBG scintigraphy were classified as true positive, false positive, true negative, and false negative. Using this classification the sensitivity was found to be 78.4% in primary, sporadic pheochromocytoma, 92.4% in malignant pheochromocytoma, and 94.3% in familial pheochromocytoma giving an overall sensitivity of 87.4%. The specificity was 98.9% in primary, sporadic pheochromocytoma, 100% in malignant pheochromocytoma, and 100% in familial pheochromocytoma. The overall specificity was 98.9%. Iodine-131 MIBG scintigraphy was thus found to be a safe, noninvasive, and efficacious technique for the location of pheochromocytomas, especially for those arising from nonadrenal sites, recurring postoperatively, and exhibiting malignant metastatic disease. We find that, where available, [131I]MIBG scintigraphy is the study of choice to initiate the location of suspected pheochromocytoma.
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PMID:Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases. 286 Feb 14

Among all the radiological examination techniques, CT is today, besides scintigraphy, the method of choice as far as the detection of functional adrenal lesions is concerned. In primary aldosteronism, CT classification of the syndrome is based on the detection of an adenoma which can be reliably detected in adenoma sizes up to 8-10 mm. Thus, 70 to 80% of Conn's syndromes can be classified. In adrenal Cushing's syndrome, the distinction between adenoma and carcinoma of the adrenal gland is up to CT and can usually be easily made due to the characteristic morphology of each type of lesion. In case of a typical adrenal or juxtaadrenal tumor location, detection of a pheochromocytoma is likewise easy. In ectopic and multiple pheochromocytomas or such as occur as part of a MEN-syndrome, the situation is quite different. If lesions of the adrenal gland are found by accident in examinations otherwise indicated, the question arises whether the process is malignant or benign. In this respect, all the traditional imaging methods, including CT, involve a considerable factor of uncertainty, especially if a malignant tumor is anamnestically known and the question of metastases arises. According to recent information, MR-imaging seems to be advantageous concerning this difficult differential diagnosis.
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PMID:[Radiological diagnosis of adrenal gland diseases]. 287 94

While 131I-meta-iodobenzyl guanidine (131I-MIBG) scanning has made possible the scintigraphic visualization of pheochromocytoma and neuroblastoma, an accumulation of this agent has recently been reported in medullary thyroid cancer. We present the case of a patient with Sipple's syndrome (multiple endocrine neoplasia type IIa), in whom we were able to identify distant metastases and local invasion of medullary thyroid cancer as well as primary thyroid tumour and right adrenal pheochromocytoma, using 131I-MIBG scans. This case highlights the usefulness of 131I-MIBG in the detection of metastatic medullary thyroid cancer and suggests that this agent may also be of therapeutic use in the treatment of tumours.
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PMID:Detection of metastatic medullary thyroid cancer with 131I-MIBG scans in Sipple's syndrome. 287 28

Metastatic pheochromocytoma, a rare complication of pregnancy, was managed from 30 weeks' gestation until delivery three weeks later with a combination of alpha-adrenergic blockade (Minipres) beta-adrenergic blockade (Timolol), and dopamine synthesis inhibition (Demser). The biophysical parameters of fetal heart rate (FHR) baseline, variability, and reactivity, as well as fetal breathing movements, body movements, tone, and amniotic fluid volume were followed sequentially during this period. A 1450-g growth-retarded infant, who subsequently did well, was delivered by cesarean section; the mother received combined surgical and medical therapy for her metastatic disease in the postpartum period. The initial fetal biophysical alteration observed was a reduction in mean FHR baseline rate; further biophysical test abnormalities appeared only after overt fetal compromise was evident. Sequential multiple parameter biophysical testing in such circumstances appears to be a valid and valuable approach to antepartum management.
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PMID:Metastatic pheochromocytoma in pregnancy and fetal biophysical assessment after maternal administration of alpha-adrenergic, beta-adrenergic, and dopamine antagonists. 287 38

From 1979 to 1986, seven patients with multiple endocrine neoplasia (MEN) type IIa and three with type IIb were treated. Nine had a C-cell carcinoma, one had C-cell hyperplasia. None had hyperparathyroidism. Three patients had multiple mucous neuromas. Six patients had proven pheochromocytoma: adrenalectomy was performed in these six (unilateral or bilateral depending on whether the tumor was uni- or bilateral). All ten patients had a total thyroidectomy--three later required neck dissection for regional lymphnode metastases. One patient died from the consequences of diffuse liver metastases of a C-cell carcinoma. Extensive family screening is necessary with patients who have MEN type II, in order to discover early any occult disease carriers. In addition, MEN type II should be excluded in all patients who have C-cell carcinoma, pheochromocytoma or hyperparathyroidism.
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PMID:[Type II multiple endocrine neoplasms. Diagnosis, therapy and prognosis]. 288 91

Since 1975, 10 families with the multiple endocrine neoplasia (MEN)-2A syndrome and five with the MEN-2B syndrome, making a total of 101 patients, have been identified in The Netherlands. Twenty-three of the MEN-2A patients died before the start of the screening program. The average age of the patients whose death was due to pheochromocytoma (n = 11) or medullary thyroid carcinoma (n = 12) was 34.9 and 49.2 years, respectively. Eighty-seven patients with the MEN-2A syndrome and eight with the MEN-2B syndrome underwent thyroidectomy for C-cell hyperplasia and/or medullary thyroid carcinoma. Eighteen patients had signs or symptoms caused by MEN-2A (group A), 60 were relatives of these patients who had been found to be affected at the first screening of the family (group B), and nine relatives had had negative screening results that later became positive (group C). Five patients had signs or symptoms due to MEN-2B (group A) and three were relatives of these patients who had been found to be affected at the initial screening (group B). To assess the effect of screening, we compared these groups with respect to the occurrence of metastatic medullary thyroid carcinoma at thyroidectomy and the results of the postoperative calcitonin tests. Among the MEN-2A families, 72 percent of group A, 33 percent of group B, and none of group C were found to have metastatic medullary thyroid carcinoma at surgery. In the MEN-2B families, all five patients in group A and one of the three patients in group B had metastatic disease. The "cure rates" in these three groups with MEN-2A, as determined by stimulated calcitonin assessment, were 11, 57, and 100 percent, respectively. One of the five patients with MEN-2B in group A and two of the three patients in group B showed normalization of the stimulated calcitonin value after surgery. From these results, it may be concluded that screening can lead to the detection of medullary thyroid carcinoma in an earlier stage, which in turn may permit curative treatment and improvement of both prognosis and life expectancy. The need for supervision of affected families by central registration to promote periodic examination and to guarantee the continuity of such screening is discussed.
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PMID:Multiple endocrine neoplasia syndrome type 2: the value of screening and central registration. A study of 15 kindreds in The Netherlands. 289 Mar

The scintigraphic appearance of the neoplasms in multiple endocrine neoplasia type 2B (MEN-2B) and the interpretations of the image patterns are described. An 18-year-old male patient with the MEN-2B syndrome underwent TI-201 imaging that showed concentrations of TI-201 in the primary medullary thyroid carcinoma (MTC) tumor and in cervical lymph node metastases. After total thyroidectomy and lymph node dissection, the TI-201 image was normal. Catecholamine levels in the blood and urine were only borderline elevated. Yet, greater than normal concentrations of I-131 metaiodobenzylguanidine (I-131 MIBG) were present in both adrenal glands. Computed tomography of the abdomen showed normal adrenal glands. These results were consistent with the diagnosis of adrenal medullary hyperplasia, a precursor of pheochromocytoma. No operation was indicated to remove the adrenal glands. Imaging with TI-201 appears to be useful in identifying sites of MTC in patients with the MEN-2B syndrome. I-131 MIBG imaging, in conjunction with computed tomography of the adrenal glands and appropriate catecholamine measurements, should be performed in patients with the MEN-2B syndrome to determine the status of the adrenal medullae, which then may be classified as normal, hyperplastic, or tumorous with pheochromocytoma.
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PMID:Scintigraphic portrayal of the syndrome of multiple endocrine neoplasia type-2B. 290 89


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