Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of a patient with a metastatic ACTH (adrenocorticotrophic hormone)-producing gastric carcinoid APUD (amine precursor uptake and decarboxylation) tumor is presented. The patient presented with Cushing's syndrome and very high levels of ACTH. The Cushing's syndrome was controlled medically, and when the patient deteriorated clinically she received treatment with a combination of BCNU, adriamycin, and ftorafur. She responded dramatically with marked clinical improvement and normalization of plasma ACTH levels. The current status of the APUD cell concept is discussed, with its overall implications for treatment of this group of malignancies.
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PMID:Successful therapy of an ACTH-producing gastric carcinoid APUD tumor: report of a case and review of the literature. 626 Mar 30

We performed selective venous catheterization and sampling for ACTH in six patients with ACTH-secreting pituitary adenomas (Cushing's disease) and four patients with occult ectopic ACTH-secreting neoplasms. In five patients with Cushing's disease in whom the inferior petrosal sinus could be catheterized, ACTH levels were unequivocally higher than simultaneous peripheral values: The ratio was greater than 2.0, with a range of 2.2 to 16.7. In contrast, the inferior petrosal sinus-to-peripheral ACTH ratio in three patients with ectopic ACTH secretion was less than 1.5. In the fourth patient, an arteriovenous gradient of 6.8 was shown 2 years before a bronchial carcinoid tumor was clinically apparent. Central-to-peripheral ACTH ratios at the level of the jugular bulb and jugular vein were not diagnostic. We conclude that selective venous ACTH sampling from the inferior petrosal sinus is a reliable and useful aid in the differential diagnosis of Cushing's syndrome when standard clinical and biochemical studies are inconclusive.
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PMID:Selective venous sampling for ACTH in Cushing's syndrome: differentiation between Cushing disease and the ectopic ACTH syndrome. 626 40

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

Clinical and laboratory data, histologic, electron microscopic and immunocytochemical findings of the tumors of eight patients suffering from Cushing's syndrome and of one patient with hypercalcemia are described. The unlabeled antibody enzyme method was used for the detection of insulin, glucagon, somatostatin, pancreatic polypeptide, corticotropin, beta-lipotropin, calcitonin, parathyroid hormone, and gastrin. Ectopic Cushing's syndrome was caused by pancreatic endocrine tumors, medullary thyroid carcinoma, a bronchial, a gastric and a thymic carcinoid, and a carcinoid of the mediastinum. Hypercalcemia in one patient was related to a pancreatic endocrine tumor. After surgery the clinical symptoms disappeared in two patients, but persisted or relapsed in five patients. ACTH-immunoreactivity could be demonstrated in six of eight tumors; calcitonin-immunoreactivity was found in the tumor of the patient suffering from hypercalcemia. ACTH-immunoreactivity could be localized to secretory granules by immunoelectron microscopy, and the presence of ACTH and beta-LPH in the same tumor cells could be shown in one pancreatic tumor. A combination of production of orthotopic and ectopic hormones was found in one, and secretion of two ectopic hormones was detected in another pancreatic endocrine tumor.
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PMID:Ectopic hormone production by endocrine tumors: localization of hormones at the cellular level by immunocytochemistry. 627 90

A study was undertaken of five patients with Cushing syndrome due to adrenocorticotropin (ACTH) production by thymic carcinoid tumor (primary mediastinal APUDomas), including two recent patients examined by computed tomography (CT) of the chest. Plain roentgenography of the chest initially failed to detect tumor in four of the five patients, while CT of the chest yielded definitive diagnostic information in both patients in whom it was employed. For one of these patients, a mediastinal tumor could be seen retrospectively on plain roentgenograms of the chest, although it had been missed on the first examination. One of the tumors appeared to be partially calcified on CT scan, a finding not previously reported. Blastic osseous metastasis, which is common when malignant carcinoid tumors spread to bone, was seen in one patient. Our data suggest that in patients with suspected ectopic ACTH production, CT scanning of the mediastinum should be performed early in order to avoid delay in diagnosis of an ACTH-secreting carcinoid tumor of the mediastinum.
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PMID:Roentgenologic diagnosis of primary corticotropin-producing carcinoid tumors of the mediastinum. 627 65

Bromocriptine (0.13-13 microM) significantly inhibited ACTH secretion in a dose-dependent manner when added to cell cultures of a human corticotrophic adenoma for 24 h. Haloperidol (13 microM), but not serotonin (13 microM), blocked this inhibition but had no significant effect when added alone. In addition, dopamine (10 microM) reduced ACTH secretion during a 4-h incubation, whereas serotonin (0.01-10 microM) was ineffective. An ectopic ACTH secreting lung carcinoid was non-responsive to doses of bromocriptine up to 13 microM. These results demonstrate a direct suppressive action of bromocriptine on a human pituitary corticotrophic adenoma through dopaminergic rather than serotoninergic mechanisms.
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PMID:Bromocriptine suppresses ACTH secretion from human pituitary tumour cells in culture by a dopaminergic mechanism. 627 51

A patient with a thymic carcinoid tumour causing ectopic ACTH syndrome is presented. The case illustrates the rapid development of the clinical and laboratory findings often associated with ectopic secretion of ACTH, including severe proximal myopathy, emotional lability, and hypokalaemic alkalosis. Interpretation of conventional tests of pituitary-adrenal function was complicated by intermittent secretion of ACTH by the tumour. The results of selective venous sampling for ACTH ruled out pituitary ACTH hypersecretion and were suggestive of a thymic source; computerized tomography of the chest localized the tumour. In vivo and in vitro investigations confirmed excessive ACTH production by the tumour, and surgical resection plus radiotherapy has resulted in resolution of the syndrome. The diagnostic problems created by intermittent secretion of ACTH by these tumours and the pre-operative and post-operative medical management of these patients are discussed.
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PMID:A case of ectopic ACTH syndrome: diagnostic difficulties caused by intermittent hormone secretion. 628 Apr 27

A 51-year old man presented with the classical features of Cushing's syndrome which had evolved over the previous 5 yr, and was found to have ACTH secretion from an atypical thymic carcinoid tumor. Tumor extract, assayed under conditions designed to prevent artefactual generation of peptides, was found to contain a wide variety of immunoreactive hormones including ACTH, alpha-MSH, CLIP, beta-endorphin and met-enkephalin. The ACTH-related peptides were probably derived from a common precursor, pro-opiocortin, but the presence of met-enkephalin suggests the production of a separate type of precursor molecule. The tumor was locally invasive and, depsite subtotal excision and radiotherapy, continued to secrete large amounts of ACTH. Hypercortisolism was controlled longterm with pharmacological adrenal blockade and steroid replacement.
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PMID:Ectopic secretion of ACTH and met-enkephalin from a thymic carcinoid. 628 29

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


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