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)

Bronchial carcinoid tumors are usually indolent, slow-growing tumors with an excellent prognosis. However, even typical carcinoids can metastasize to regional lymph nodes or to distant sites. Atypical carcinoids tend to behave more invasively with more frequent nodal and distant metastases. Despite this, long-term survival can be expected as many tumors grow and spread slowly. At the end of the spectrum are the highly aggressive small cell carcinomas which have a very poor prognosis despite aggressive chemotherapy. Clinically, carcinoid tumors are frequently asymptomatic. Symptoms are most frequently due to obstruction (pneumonia, 'asthma', coughing) or bleeding. Carcinoid syndrome is seen infrequently and usually signifies metastatic disease. Cushing's syndrome is occasionally seen in association with these tumors. The treatment of carcinoid tumors is surgical. Resection should be complete and encompass the regional lymph nodes.
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PMID:Bronchial and thymic carcinoid tumors: a review. 769 41

Paraneoplastic manifestations are signs and symptoms observed in patients with cancer, distant from the tumour or its metastases and not caused by invasion, obstruction or bulk mass. In children with cancer, paraneoplastic manifestations are rare and distinct from those observed in adults. Knowledge about paraneoplastic manifestations can be of great clinical importance because they may be the presenting sign of a tumour or its recurrence and hence facilitate early diagnosis. In contrast, they sometimes mask the symptoms of a tumour and cause diagnostic delay. In this review, paraneoplastic manifestations in children are described, including hypercalcaemia, Cushing syndrome, precocious puberty, opsoclonus/myoclonus, acquired von Willebrand disease, watery diarrhoea syndrome, and hypertension. The mechanisms causing these manifestations are also discussed.
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PMID:Paraneoplastic manifestations in children. 784 90

Adrenal diseases are uncommon in older patients but can present insidiously. Adrenal masses are often discovered incidentally on abdominal CT scans and are usually benign, but they should be assessed for hormone production. Cushing's syndrome, or cortisol excess, is usually due to exogenous corticosteroids or a hormonally active tumor. The overnight dexamethasone suppression test is an excellent screening test. Adrenal insufficiency can be caused by hemorrhage, tuberculosis, or metastatic cancer. Maintaining a high index of suspicion of hidden adrenal disease, the primary care physician should apply an efficient approach to the diagnosis and initial management of adrenal diseases in older patients.
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PMID:Adrenal disorders: know when to act and what tests to give. 802 Jul 60

We present a patient with a poorly differentiated squamous cell carcinoma of the left lower lobe, who developed Cushing's syndrome. Adrenocorticotrophic hormone (ACTH) and cortisol levels in the blood were extremely elevated, but immunostaining for ACTH and corticotrophin releasing factor (CRF) of the primary tumour and metastases was negative. The ectopic Cushing's syndrome was probably caused by CRF-like production.
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PMID:Ectopic Cushing's syndrome in a patient with squamous cell carcinoma of the lung due to CRF-like production. 819 14

In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children.
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PMID:[Cushing syndrome in children]. 823 97

In the last 20 years, eleven children with adrenocortical functional tumors were treated in the National Children Hospital of Costa Rica. There were nine females and two males and their ages ranged from nine months to 14 years. Eleven patients had features of virilism, five had stigmas of Cushing's syndrome and three hyperaldosteronism. The clinical diagnosis was established given the symptoms, hormonal tests and radiological and imagenological studies. The histologic diagnosis was carcinoma in six by clinical picture in one, and were adenoma in four. Three patients had regional and distant metastases. Four patients with carcinoma were treated by surgery and five received chemotherapy, two of them in presurgical stage, and four received radiotherapy. Two patients with carcinoma are alive and had no evidence of tumor recurrence ten and six years after diagnosis. Five are dead, two of them after partial response to chemotherapy. The four patients with adenoma were cured by complete surgical tumor resection, furthermore one of them received chemotherapy because there was not sure of his histologic benign condition. It is necessary more studies in use of chemotherapy in treatment of this tumors but in our experience CFM, VCR, Epi and Actin is a regimen that appears to be an active combination for the treatment in presurgical stage of adrenal cortical carcinoma.
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PMID:[Functioning tumors of the adrenal cortex in children. Clinical and therapeutic considerations on 11 cases]. 837 47

Carcinoid of the thymus rarely occurs during childhood. The authors identified eight cases in patients younger than 17 years of age. All were associated with Cushing syndrome. Adrenocorticotropic hormone (ACTH) produced by the tumor may be released intermittently, delaying the findings of Cushing syndrome. The authors describe a case of ectopic ACTH production in a teenaged boy who had longstanding hyperpigmentation, increased ACTH levels, and normal cortisol levels. Magnetic resonance imaging of the pituitary had normal findings. Subsequently, severe Cushing syndrome developed. Computed tomography (CT) scans of the chest showed a mediastinal mass that proved to be a thymic carcinoid. The lesion was inoperable. Radiation and chemotherapy were of limited benefit. Metyrapone was used to control hypercortisolism. The patient died with extensive metastases 6 years after initial presentation. CT scans of the chest should be performed in an attempt to localize ectopic ACTH-producing tumors. Surgical excision of the lesion is the treatment of choice. Control of hypercortisolism is essential.
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PMID:Adrenocorticotropic hormone--producing thymic carcinoid in a teenager. 838 Jan 12

A 62-year-old male with small cell lung cancer (SCLC) associated with Cushing's syndrome and diabetes insipidus (DI) is reported. The patient was referred to our hospital for treatment of SCLC. A diagnosis of paraneoplastic Cushing's syndrome was made on the basis of an elevated serum ACTH (623.5 pg/ml) level, elevated excretion of urinary 17-OHCS (18.01 mg/day), obesity, hypertension, hyperglycemia, persistent hypokalemia, alkalosis, and no history of diabetes mellitus. He was also diagnosed as having DI based on polyuria and polydipsia, low specific gravity of the urine (1.007-1.010), low serum ADH (1.4 pg/ml) level, normal plasma osmolarity (29 mOsm/kg H2O), and the results of water deprivation test. DI and a left visual field defect was suggestive of metastasis to the pituitary region, but no lesion was detected by either CT scan or MRI scan. The patient failed to show a good response to intensive chemotherapy, and died of the tumor five months after commencing chemotherapy. Post-mortem examination revealed metastases to the hypothalamic-neurohypophyseal region, lungs, liver, adrenal glands, bone, bone marrow, and hilar and mediastinal lymph nodes.
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PMID:[A case of small cell lung cancer associated with diabetes insipidus and Cushing's syndrome]. 839 May 89

The radiological features of 14 patients with Cushing's syndrome due to 'occult' ectopic ACTH syndrome have been reviewed. In 11 of the 14 patients (79%), the ACTH-producing tumour was located in the thorax. Bronchial carcinoid tumours (eight patients) were the single most common source of 'occult' ectopic ACTH production with thymic carcinoid tumours (two patients) and mediastinal metastases from a medullary carcinoma of the thyroid gland (one patient) accounting for the other intrathoracic tumours. The tumours were usually small with five of the eight bronchial carcinoid tumours measuring between 4 and 10 mm in diameter. Since it remains difficult to distinguish between pituitary-dependent Cushing's disease and 'occult' ectopic ACTH-dependent Cushing's syndrome both clinically and biochemically, the role of radiology remains vital.
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PMID:The radiological investigation of occult ectopic ACTH-dependent Cushing's syndrome. 839 21

An autopsy case of ACTH-producing thymic carcinoid with Cushing's syndrome is reported. The patient was a 63-yr-old man with multiple bone metastases from an undetermined primary site. Hyperpigmentation was observed at the terminal stage. The plasma levels of ACTH, cortisol, chromogranin A, and urinary 17-hydroxy-corticosteroids were extremely high, and ectopic ACTH-producing neuroendocrine tumor was diagnosed. In addition, plasma CRH was high. Autopsy revealed that the patient had primary thymic carcinoid with extensive metastases. Remarkable hyperplasia of the adrenal cortexes and Crooke's hyaline degeneration of the pituitary gland were consistent with Cushing's syndrome by ectopic ACTH production. There were multiple CRH-producing cells without degenerative changes in the hypothalamus. The tumor cells were immunoreactive to ACTH, CRH, and the cleavage enzyme PC2. POMC messenger ribonucleic acid and PC2 messenger ribonucleic acid were detected in the tumor cells by an in situ hybridization method. Expression of PC2 was considered to induce hyperpigmentation by producing alpha MSH. Despite hypercortisolism and ectopic production of CRH by the tumor cells, hypothalamic CRH cells were not atrophic. This case is a good example to demonstrate the correlation between CRH and the hypothalamo-pituitary-adrenal axis as well as hyperpigmentation in Cushing's syndrome.
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PMID:Expression of prohormone convertase, PC2, in adrenocorticotropin-producing thymic carcinoid with elevated plasma corticotropin-releasing hormone. 855 Jul 83


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