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)

We performed combination therapy with cyclophosphamide, Vincristine and Dacarbazine (CVD) regimen and transarterial embolization (TAE) in 2 cases of malignant pheochromocytoma with metastases. Case 1: 59-year-old female. After primary left adrenal lesion had been removed, recurrence at the left renal hilar region and metastases to the right iliac bone and 5th cervical vertebra occurred. We took 3 courses of CVD regimen after TAE for the lesions in the right iliac bone. Her endocrinological data has been normal for more than 1 year after treatment. Case 2: 29-year-old male. Total cystectomy, ileal conduit and pelvic lymphadenectomy had been performed for the primary lesion of the urinary bladder. 2 years after the 1st operation, metastases to the right obturator nodes and multiple bones occurred. We gave 3 courses of CVD regimen followed by TAE for the lesions in the right obturator nodes. Just after treatment, we could stop insulin and reduce anti-hypertension drugs, but the effect of treatment was temporary. In conclusion, combination of CVD regimen and TAE is effective for malignant pheochromocytoma with metastases.
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PMID:[Treatment of malignant pheochromocytoma by combination of CVD regimen and transarterial embolization]. 187 77

We reviewed the results of fine needle biopsy of the adrenal glands guided by ultrasonography or CT in 56 patients. The final diagnoses, obtained at operation, autopsy or follow-up were: metastasis (n = 22), adenoma (n = 21), adrenal cyst (n = 6), hematoma (n = 3), lymphoma (n = 1), pheochromocytoma (n = 1), lymph node (n = 1), and amyloid mass (n = 1). Sufficient cytologic material was obtained in 96.4% (54/56). The overall accuracy to differentiate benign from malignant disease was 85.7% (48/56), 2 were false-negative, one was false-positive. The biopsy was inconclusive ("possibly malignant") in 3 patients, 2 of whom had an additional cutting needle biopsy yielding a correct positive finding. No complications occurred. We conclude that in disseminated malignant disease with suspected adrenal metastases diagnostic results can be obtained with guided fine needle biopsy. Biopsy in primary adrenal lesions is helpful, especially if the aspirate of the lesion turns out to be composed of something other than adrenal cells.
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PMID:Accuracy of adrenal biopsy guided by ultrasound and CT. 191 Sep 90

Medullary thyroid carcinoma, comprising approximately 7% of thyroid carcinoma, produces calcitonin, which can be monitored by immunoassay for diagnosis, at preclinical stages, and for persistent disease and its extent. It presents as sporadic and hereditary forms. The latter consists of the multiple endocrine neoplasia (MEN)-2A syndrome, which includes pheochromocytomas and hyperparathyroidism in some families, and the MEN-2B syndrome, which consistently includes mucosal neuromas and somatic features. The carcinoma, especially the MEN-2B variety, is more aggressive than well-differentiated thyroid carcinoma. After the presence and management of a possible pheochromocytoma is resolved, treatment is by total thyroidectomy, the MEN-2 syndromes always indicating bilateral involvement. Gross evidence of medullary thyroid carcinoma is associated with metastases to regional lymph nodes, justifying removal of lymph nodes in the central neck, anterior superior mediastinum, and lateral neck. At operation, attention is given to preservation of parathyroid glands but also to removal of hyperplastic parathyroid glands; subtotal parathyroidectomy usually is needed if clinical hyperparathyroidism is evident. Diagnosis at the preclinical stage, C-cell hyperplasia, permits total thyroidectomy. Lateral cervical lymph node dissection is determined by biopsy of midjugular lymph nodes. In this situation, serum calcitonin levels are usually normal after operation, indicating cure. However, for palpable medullary thyroid carcinoma, serum calcitonin levels are often elevated after appropriate neck surgery. In this event, scanning techniques are used to monitor patients, and reoperation is performed if localization of medullary thyroid carcinoma is achieved. The mediastinum is particularly observed for recurrence. Reoperation is justified for recurrence in the neck and mediastinum. Early diagnosis and monitoring permits long-term survival. In the future it is anticipated that diagnostic genetic techniques will provide definitive and early diagnosis in the hereditary form, permitting earlier treatment with assurance of cure.
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PMID:Surgical treatment of medullary carcinoma of the thyroid. 197 81

Eight Sipple's syndrome patients from four families have been reviewed. One family had the largest number of members with pheochromocytoma and/or medullary thyroid carcinoma. A total of 82 cases (47 females and 35 males) collected from the Japanese literature during the period 1960-1989, are also reviewed. The ages ranged from 22 to 73 (median 41) years, 13%, being over the age of 60 years (elderly patients). Pheochromocytoma occurred bilaterally in 72% of cases. With Sipple's syndrome, a relatively high urinary excretion of the epinephrine fraction and a high content of epinephrine in the tumor tissues were thought to be characteristic. Seventy-three patients underwent adrenalectomy with a successful outcome for 62 (85%). There needs to be careful periodic follow-up after unilateral adrenalectomy. Medullary thyroid carcinoma (MTC) also occurred bilaterally and was multicentric in 66% of cases, and was often found to be metastasized to the cervical lymph nodes at the time of surgery (35%). Parathyroid disease was found in 22% of cases: parathyroid adenoma in nine, hyperplasia in 10. Fourteen patients (17%) died of surgical complications, hypertensive crisis caused by excessive catecholamine release and/or widespread MTC metastases.
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PMID:Analysis of eight Sipple's syndrome patients and review of eighty-two cases from the Japanese literature. 198 Sep 32

Surgical treatment of hepatic metastases is predominantly aimed at "curative" resection. This can be achieved in about 20% of colorectal secondaries, and is associated with a 30-40% 5-year survival. One to three metastases in the absence of extrahepatic disease are regarded a clear indication to resection. Among patients with non-colorectal malignancies, occasional long term survival was reported in leiomyosarcoma, breast cancer, and renal cancer metastases, respectively. Endocrine tumors such as carcinoid, gastrinoma, or pheochromocytoma, are different because of their remarkable symptoms along with a protracted natural history. Quality of life may be considerably improved here by even non-radical debulking. The vast majority of patients, however, ist not suitable to undergo hepatic resection. Palliative therapeutic options involve hepatic artery ligation or embolization, cryo-surgery and percutaneous laser coagulation, and various types of regional chemotherapy. These methods may enable a temporary relief of symptoms, but no significant impact on survival time, and no true long term benefit has been proven. Prospective randomized trials against combined treatment as well as untreated patients are required for a more meaningful judgement and improved effectiveness.
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PMID:[Surgical interventions in liver metastases]. 198 54

We report our experience with the management of 7 primary nonrenal parenchymal malignancies with vena caval tumor thrombus. Included are 3 cases of adrenal cortical carcinoma and 1 each of transitional cell carcinoma, embryonal cell testicular carcinoma, pheochromocytoma and primary small cell carcinoma of the lung with metastases to the kidney. Surgical treatment and followup are presented, as well as a review of the literature. An aggressive surgical approach is warranted because prolonged survivals free of disease are possible.
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PMID:Management of primary nonrenal parenchymal malignancies with vena caval thrombus. 198 10

The efficacy and safety of m-[131I]iodobenzylguanidine ([131I]MIBG) were assessed in 15 patients with malignant pheochromocytomas in a nonrandomized, single arm trial, in which patients were treated with [131I]MIBG (SA, 740 megabequerel/mg) every 3 months. Seven of these patients had bone and soft tissue metastases, 4 had only soft metastases, and 4 had only bone metastases. The follow-up period ranged from 6-54 months; the number of doses ranged from 2-11, with 2.9 (78.4 mCi) to 9.25 gigabequerel (GBq) (250 mCi)/administration and a cumulative activity from 11.1-85.90 GBq (300-2322 mCi). The absorbed cumulative dose in tumors ranged from 12-155 Gy. A beneficial effect of the treatment was observed in 9 patients (60%). No complete remission of the disease was observed. Seven patients died during the study, among whom 4 never responded to the treatment. Seven had hormonal responses (4 complete and 3 partial), with a duration ranging from 5-48 months. Among these patients, 4 relapsed, and 3 died within 3 months. Five patients had partial tumoral responses mainly located in soft tissues and for a duration ranging from 29-54 months. All patients with a hormonal response had objective improvement in clinical status and blood pressure. There was no clear-cut relationship between the cumulative dose and the responses. The main side-effect observed in 1 patient with widespread bone metastases after three doses (12.9 GBq) was a pancytopenia, which resolved after treatment was discontinued. This study suggests that repeated [131I]MIBG treatment could be effective in patients with advanced malignant pheochromocytoma.
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PMID:Use of m-[131I]iodobenzylguanidine in the treatment of malignant pheochromocytoma. 199 14

Fifteen patients with medullary carcinoma of the thyroid (MCT), who had persistently elevated levels of serum calcitonin (CT) and carcinoembryonic antigen (CEA) after total thyroidectomy, were studied in order to localize the sites of the recurrent disease. Routine diagnostic examinations, including ultrasonography (US) and computed axial tomography (CAT), were carried out in all the cases. Scintigraphy with radio-iodinated metaiodobenzylguanidine ((131I)-MIBG) was performed in 13 cases; selective venous catheterization (SVC) to reveal a gradient of CT levels was performed in 12 cases. Ten patients underwent both (131I)-MIBG scintigraphy and SVC. US and CAT revealed the sites of recurrent tumor in only 4 out of the total 15 patients. SVC in basal conditions showed the presence of small metastases in 2 cases, and after intravenous stimulus with pentagastrin in 4 others. The MIBG scan showed metastatic foci of sporadic MCT in 2 patients, residual medullary thyroid tissue in 4 others, and a pheochromocytoma in a previously undiagnosed patient with Sipple's syndrome. More particularly, MIBG scan and SVC showed the localization of residual or metastatic tumor in 10 cases. In all 10 cases, results of the MIBG scan and SVC were confirmed as true positive by subsequent surgery and histopathologic examination.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:(131I)meta-iodobenzylguanidine scintigraphy and selective venous catheterization after thyroidectomy for medullary thyroid carcinoma. 204 83

Carcinoid tumors of the midgut type are slowly growing neoplasms which often present clinically and histologically pronounced fibrosis around the tumors. Cryosections from 41 neuroendocrine tumors (31 midgut carcinoid tumors, 8 endocrine pancreatic carcinomas, 1 parathyroid carcinoma, and 1 pheochromocytoma) and 22 nonneuroendocrine carcinomas were examined for the presence of platelet-derived growth factor (PDGF) beta-receptor by immunohistochemistry using the monoclonal antibody PDGFR-B2. Twenty midgut carcinoid tumor tissues (66%) and 4 endocrine pancreatic carcinomas (50%) and the parathyroid carcinoma stained positively with the antibody. In contrast, only 2 nonneuroendocrine tumor tissues (10%) were stained, and the staining in these cases was weak. The immunoreaction in the carcinoid tumors was observed in connective tissue cells adjacent to tumor cell clusters but not in the tumor cells themselves. The degree of positive PDGF beta-receptor expression in the carcinoid tissues seems to correlate positively with the presence of macrophages as determined by the monoclonal antibody anti-Leu-M5, but not with other infiltrated lymphocytes identified with the monoclonal antibody anti-Leu-4, or with anti-HLA-DR antibodies. Stromal cells adjacent to tumor cells, including small capillaries, stained more strongly than the stromal cells which were distant from tumor cell clusters. Furthermore, carcinoid tumor metastases from lymph nodes as well as from liver showed stronger immunoreactivity in the stromal cells with the PDGF beta-receptor antibody than the corresponding primary tumors. Our data suggest that carcinoid tumor cells may directly or indirectly induce expression of PDGF beta-receptor on adjacent stromal cells in the tumor tissue, which may contribute to the fibrosis that is often seen around carcinoid tumors.
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PMID:Expression of platelet-derived growth factor beta-receptors on stromal tissue cells in human carcinoid tumors. 215 46

Widespread use of contemporary imaging techniques (ultrasound, computerized axial tomography, and magnetic resonance imaging scans) have led to the incidental discovery of asymptomatic adrenal neoplasms with increasing frequency. Patients with such adrenal "incidentalomas" typically have no clinical manifestations of adrenal cortical hyperfunction at the time of discovery. We have studied 122 patients with asymptomatic adrenal masses ranging in size from 2 to 7 cm in diameter from 1978 to 1988. Selected patients, after adrenal metastases, pheochromocytoma, myelolipomas, and cysts were ruled out, were further evaluated for adrenal cortical hyperfunction by measuring urinary 17-hydroxysteroids, 17-ketosteroids, and free cortisol, serum A.M., P.M. cortisol, and plasma ACTH levels. These values were also measured before and after dexamethasone suppression. NP-59 adrenal scintiscans were performed on all patients. Six patients were identified with sub-clinical Cushing's syndrome. Baseline cortisol levels were normal in each of these patients. Loss of diurnal rhythm appeared to be the most sensitive indicator of abnormal adrenal cortical function. When adrenalectomy is performed in such patients, especially when contralateral adrenal gland suppression is evidenced by NP-59 scanning or other biochemical assessment, perioperative steroids should be administered in a manner similar to that used for patients with symptomatic Cushing's syndrome. Unilateral adrenalectomy in a patient with an asymptomatic adrenal adenoma, insufficiently studied, may result in Addisonian crisis.
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PMID:Sub-clinical Cushing's syndrome in patients with adrenal gland incidentalomas. Pitfalls in diagnosis and management. 216 35


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