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)

A case of malignant ACTH-producing hypophyseal tumor with serial CT scan and histological investigation is discussed. The neoplasm became evident with a Cushing's syndrome and was treated with adrenalectomy and radiotherapy. Nelson's syndrome subsequently developed due to histologically atypical adenoma. Subarachnoid metastases occurred after 4 years, finally causing a fatal intracranial hemorrhage.
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PMID:ACTH-producing hypophyseal carcinoma monitored by computed tomography. 609 73

Seven female patients with a pancreatic islet cell tumor and Cushing's syndrome are described. Five patients initially had symptoms and signs of hypercortisolism, and two presented with an abdominal mass and had subsequent development of the syndrome. The tumors ranged in size from 2 to 12 cm in diameter, and all were argyrophilic with the Grimelius technique. Four cases had positive immunostaining for adrenocorticotropic hormone; other hormones were identified in four cases. Six of the patients were dead at the time of this report: five with metastatic tumor within 4 years of diagnosis and one 19 years after diagnosis with hepatic metastases of undetermined origin. One patient was alive with hepatic metastases 20 years after operation. Among the 42 known cases of pancreatic islet cell tumor and Cushing's syndrome, the 5-year survival was 16%.
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PMID:Pancreatic islet cell tumor associated with Cushing's syndrome. 609 31

The observation of two cases of hyperworking corticosuprarenal carcinomata with diffuse metastases, has induced the authors to examine this problem on the base of the most actual bibliography of the last years. They explain in detail the 2 cases, that are a feminizing tumor in a 55 years old man and a Cushing syndrome with hypertension in a 18 years old women. The peculiar characteristic are in the first case the clinical rareness of feminizing syndrome from suprarenal carcinoma and in the second case the histopathological particularity of splenic metastases. In regard to therapy for this particular tumor, the AA. incline for the surgical removal of tumor, that can induce, in the most favourable cases, a partial or total reduction of endocrine symptomatology and for the giving in great doses of o,p'-DDD from the immediate post-operating period.
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PMID:[Associated role of surgery and antimitotic o, p'-DDD treatment in hyperfunctioning adrenal cancers with diffuse metastases]. 616 74

We report an 18-yr-old youth with a metastatic foregut carcinoid tumor, Cushing's syndrome, and hypersomatotropic gigantism. Administration of cyproheptadine caused a dramatic fall in urinary cortisol excretion and plasma ACTH levels associated with clinical remission of the Cushing's syndrome. GH secretion was not affected by cyproheptadine administration. Ectopic ACTH secretion was confirmed by RIA of tumor extracts and immunohistochemical demonstration of ACTH-containing cells in hepatic metastases. There were two sources of GH production demonstrated in this patient. Ectopic secretion of GH by the carcinoid hepatic metastases was documented by both RIA and immunohistochemical techniques. A somatotrophic pituitary tumor was also present. The histological characteristics of this tumor suggest adenomatous hyperplasia rather than de novo neoplastic change as the likely mechanism of its pathogenesis. GH releasing factor-like activity was demonstrated in extracts of plasma and in extracts of the carcinoid tumor. We conclude that cyproheptadine exerted an effect on the ectopic ACTH-producing cells but not on the ectopic GH-producing cells or on adenohypophyseal GH secretion. Production of a GH releasing factor-like activity by the carcinoid tumor may have caused the pituitary somatotrophic tumor.
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PMID:Acromegaly and Cushing's syndrome associated with a foregut carcinoid tumor. 627 Jan 70

A study of 15 cases of carcinoid tumor of the thymus, diagnosed and treated at the Mayo Clinic, revealed histopathologic features of the neoplasm that might cause it to be confused with other mediastinal tumors, both primary and secondary. The tumor was associated with ectopic ACTH production in six patients, of whom five had Cushing's syndrome. Metastasis, which occurred in 11 patients (73%), was delayed for as long as 8 years after initial diagnosis. Eleven patients underwent surgical resection of their primary thymic neoplasms, and nine of these received postoperative radiotherapy or chemotherapy or both. One patient was treated with chemotherapy alone, and three had no treatment for their neoplasms, which were discovered at autopsy. Eight patients are still alive. Of these, five have developed metastases, and only one is free of metastatic disease more than 5 years after diagnosis. In the two remaining cases, the discovery of the thymic tumor was recent and follow-up is not yet meaningful. Overall, four of the seven patients who died had proven metastatic disease. The mean survival after the appearance of extrathymic tumor in this group was 3 years. Of the five patients who are still alive with metastasis, all have survived at least 2 years since that spread of their disease.
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PMID:Primary mediastinal carcinoid tumors. 628 47

Most patients with extra-pituitary ACTH-secreting tumors die from carcinoma, but a few can benefit from operation. Of 96 patients with Cushing's syndrome, 11 probably had such tumors. There were three modes of presentation: (1) three had malignant tumors with visceral metastases initially. One (bronchial carcinoid) died without operation. Two with carcinoma (thyroid medullary and islet-cell) underwent adrenalectomy with remission, but died soon. (2) three had apparently benign tumors initially. One (appendicular carcinoid) underwent appendectomy and one (bronchial carcinoid with hilar node metastases) underwent lobectomy. Both had rapid remissions. The third (pheochromocytoma) died after resection of the tumor. (3) five patients had no obvious tumors and underwent adrenalectomy with remission. In one a benign bronchial carcinoid was removed later. Four others remain well, but without localizing signs of tumor. The main biochemical features in all were hypokalemic alkalosis and very high urinary excretion of free cortisol. Seven of the eight patients without visceral metastases are in remission from one to 15 years after operation.
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PMID:Surgical management of the ectopic ACTH syndrome. 628 49

We report the history, laboratory findings, and studies performed on a 27-yr-old patient with a metastatic parasellar adenoma of the pituitary and Cushing's syndrome. She developed intense hyperpigmentation and extraordinarily high ACTH levels after bilateral adrenalectomy in 1974. With the exception of marked hyperpigmentation, she did well on glucocorticoid replacement therapy until August 1979, when multiple hepatic nodules were observed during a cholecystectomy. Histological studies and immunoperoxidase staining indicated that these lesions were pituitary tumor metastases. What were presumed to be metastatic lesions also developed in lungs and bone. This combination of liver, bone, and lung metastases from primary pituitary tumors has not previously been reported. Immunoreactive plasma ACTH concentrations were as high as 230,000 pg/ml. Similarly, high levels of plasma immunoreactive beta MSH and immunoreactive beta-endorphin were found. High doses of glucocorticoids reduced the concentration of ACTH to one seventh to one tenth the basal level. The sensitivity of plasma ACTH to exogenous steroid administration strongly suggests that an intact intracellular mechanism for negative feedback control of ACTH secretion persisted within the tumor cells. The rapid rise in ACTH and related peptides and the development of metastases after adrenalectomy suggest that both the secretory capacity and the oncogenic potential of the parasellar tumor were chronically inhibited by glucocorticoid hormones.
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PMID:A pituitary parasellar tumor with extracranial metastases and high, partially suppressible levels of adrenocorticotropin and related peptides. 630 34

A 35-yr-old woman had Cushing's syndrome, amenorrhea, and elevated and nonsuppressible levels of urinary and plasma cortisol and urinary 17-hydroxycorticosteroids. An ovarian carcinoma with extensive metastases was found. Tumor concentrations of ACTH were much lower than those in any previously reported patient with the ectopic ACTH syndrome. Cortisol levels obtained from the venous effluent of tumor-involved ovaries were higher than that in peripheral plasma obtained at the same time. Transient postoperative normalization of plasma and urinary cortisol followed partial tumor removal. Electron microscopic study of the tumor revealed a cell type consistent with steroid-secreting cells. Postmortem examination revealed atrophy of both adrenal glands and no pituitary adenoma.
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PMID:Cushing's syndrome secondary to ectopic cortisol production by an ovarian carcinoma. 630 83

The development of radiolabeled cholesterols in 1969 as precursors of adrenocortical steroid production allowed the first noninvasive imaging of the adrenal cortices. FDA-NDA approval in 1984 should allow routine use of these agents in most hospitals. NP-59 is most commonly used in the diagnosis and management of Cushing syndrome; the second most common use is in the diagnosis of primary aldosteronism. It is also helpful in the differential diagnosis of adrenal and ovarian hyperandrogenism and hirsutism, and is the only noninvasive method of detecting unilateral adrenocortical hypofunction. The newest and most popular use is in the differential diagnosis of asymptomatic masses in the region of the adrenal gland discovered incidentally with CT scan ("incidentalomas"). In this situation, the NP-59 scan can define whether the tumor is in the adrenal gland and if it is functional or nonfunctional. We believe that, in the future, radiolabeled enzyme inhibitors might offer better diagnostic imaging of the adrenal cortex, although these agents will probably not be available for routine use for some time. Our development of a radioiodinated guanethidine analog, 131I-MIBG, has allowed differentiation of normal adrenal medullary function from bilateral adrenal medullary hyperplasia before the development of hypertension or tachycardia, diagnostic increases in plasma or urinary catecholamines, or abnormal CT scans. The search for a pheochromocytoma should begin with 131I-MIBG scintigraphy. While over 90% of primary pheochromocytomas occur in the abdomen, neither a survey of the abdomen nor the finding of a single tumor should conclude the search. Whereas the CT scan can only detect a mass, the MIBG scan not only detects the mass but proves whether the mass is a pheochromocytoma. We have detected small or recurrent pheos when all other localizing studies were normal. Present drug and X-ray therapy is relatively ineffective in treating metastatic cancer of the adrenal medulla. Preliminary results in five patients indicate that, for the first time, a complex organic molecule can carry 131I into adrenal neoplasms reproducibly and in therapeutically effective doses of irradiation. We may also be able to select which patients will respond to therapeutic doses of 131I-MIBG and which patients will not respond.
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PMID:Diagnosis of adrenal tumors with radionuclide imaging. 639 13

Adrenocortical carcinomas are very rare tumors. The diagnosis and therapeutic results in 5 female patients are reported. In 4 cases the tumor displayed biochemical and clinical features of Cushing's syndrome, while one was an adrenocortical carcinoma with hypersecretion of aldosterone only. The mean age was 53.6 +/- 8.2 years. In all four cases with Cushing's syndrome the diagnosis was established by measurement of urinary free cortisol extraction rates and excretion rates of 17-ketogenic steroids (mean 1794.5 +/- 1968 micrograms/24 h for free cortisol and 50.5 +/- 24.9 mg/24 h for 17-ketogenic steroids), while the excretion of 17-OH-steroids was in the normal range in one case with hypercortisolism. The patient with mineralocorticoid excess showed markedly elevated plasma aldosterone levels (840 pg/ml) and undetectable plasma renin activity (less than 0.2 ng/ml/3 h), but normal urinary excretion rates of 17-hydroxy- and 17-ketosteroids. The adrenal lesions were localized by intravenous pyelography or sonography and by angiographic procedures, while radioscanning by 131I-cholesterol showed no uptake of activity. Unilateral adrenalectomy was performed in four cases. After appearance of hormonally active metastases o,p'DDD was administered as an antitumor agent in 3 patients. Two of these patients died 10 and 25 months after surgery. One patient with multiple lung metastases is still alive, with a postoperative course of more than 80 months.
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PMID:[Diagnosis and therapy of hormonally active cancer of the adrenal cortex. Study of 5 cases]. 726 61


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