Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016382 (flushing)
6,387 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Carcinoid syndrome originates from metastatic carcinoid tumors localized in the gastrointestinal system, pancreas, biliary vessels, bronchi, ovaries, and testes; it is characterized by flushing, telangiectasias, diarrhea, bronchoconstriction, and fibrous endocardial plaques in the heart. Cardiac involvement is detected by echocardiography in over 50% of patients with this syndrome. Right-sided valvular heart disease occurs frequently in patients with carcinoid syndrome, involving most commonly the tricuspid and pulmonary valves. Involvement of the left-sided valves rarely occurs. Medical therapy for carcinoid heart disease includes digitalis and diuretics for congestive heart failure symptoms; the effects of carcinoid syndrome can be treated with the use of somatostatin analogues. Conventional chemotherapy has been of little beneficial value, with response rates of only 10% to 30%. The use of octreotide, a long-acting and potent somatostatin analogue, is a major advance in the management of carcinoid tumors. In addition to providing effective symptom relief in malignant carcinoid syndrome, octreotide can also be used for diagnostic purposes. Despite its expense, octreotide is the current agent of choice for the treatment of this condition. Analogues with different receptor specificities and pharmacokinetics hold promise for the future. Valve surgery is the only definitive treatment for intractable right-sided heart failure. Although cardiac surgery carries high perioperative mortality, marked symptomatic improvement occurs in survivors. Surgical intervention therefore should be considered in the appropriate patients when cardiac symptoms become severe. Balloon valvulotomy in patients with severe pulmonary artery stenosis often results in palliation of symptoms; however, surgery still is required often in these patients.
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PMID:Carcinoid Heart Disease. 1109 44

A few studies have suggested an antitumour activity of somatostatin analogues in neuroendocrine tumours (NET). The aim of this study was to evaluate the antitumour efficacy of somatostatin analogues in patients with documented progressive tumours. 35 consecutive patients with documented tumour progression were treated with somatostatin analogues. Patients were classified into two groups. In Group 1, tumours were progressing rapidly (an increase of 50% or more in the lesion surface area in 3 months) and in Group 2, tumours were progressing more slowly (an increase of less than 50% in the lesion surface area in 3 months but greater than 25% in 6 months). Treatment consisted of subcutaneous (s.c.) octreotide, 100 microg thrice daily for 17 patients, intramuscular lanreotide, 30 mg/every 14 days for 11 patients and for 7 patients both somatostatin analogues were used successively during the follow-up. Primary tumour sites were the small intestine (n=12), pancreas (n=13), lungs (n=5), and other sites (n=5). 18 patients had the carcinoid syndrome with flushing and/or diarrhoea. The median duration of treatment was 7 months. Treatment was discontinued in 3 patients due to side-effects. One patient (3%) achieved a partial response and the disease was stabilised in 20 patients (57%) for a median duration of 11 months (6-48 months). Stabilisation of patients in Group 1 was significantly less frequent at 6 months than that of patients in Group 2 (4/12 and 13/17 respectively, P<0.02). Somatostatin analogue treatment resulted in one partial response (3%) and 20 cases of stabilisation (57%) in 35 patients with progressive NET. A slow tumour growth rate before treatment is predictive of a good response to somatostatin analogues which could be considered an option for first-line treatment.
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PMID:Antitumour activity of somatostatin analogues in progressive metastatic neuroendocrine tumours. 1133 27

Carcinoids are rare tumors of enterochromaffin cells. The carcinoid-syndrome most often occurs with hepatic metastases of carcinoids and is evoked by release of serotonin and other vasoactive substances, leading to typical symptoms such as hyper- or hypotension, bronchospasm, tachycardia, diarrhoe, and flushing. A lethal perioperative "carcinoid-crisis" may occur. We report on a patient with carcinoid-syndrome due to liver metastases undergoing hemihepatectomy. For prophylaxis, the patient preoperatively received H 1- and H 2-histamine-receptor antagonists, corticosteroids, and a continuous somatostatin infusion. Besides monitoring cardiovascular variables we intermittently measured serotonin- and catecholamine concentrations. Initially increased serotonin concentration decreased during the course of anaesthesia. However, it increased again during liver resection despite Pringle's manoeuvre and was associated with a decrease in arterial pressure, systemic vascular resistance, and central venous pressure. Hypotension was treated by volume and noradrenaline infusion. Thus, despite somatostatin infusion serotonin release is still possible, especially during surgical manipulation.
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PMID:[Anaesthetic management for hemihepatectomy in a patient with carcinoid-syndrome]. 1174 74

Gastroenteropancreatic (GEP) neoplasms originate from any of the various cell types belonging to the neuroendocrine system. A general characteristic of GEP endocrine tumours is that the vast majority produce and secrete a multitude of peptide hormones and amines. Many patients with malignant metastasising tumours present clinical symptoms related to hormone hyperproduction. These include the so-called carcinoid syndrome, characterised by flushing, diarrhoea, wheezing and right heart disease, which is predominantly associated with the serotonin- and tachykinins-producing carcinoids of the midgut. Several types of syndrome associated with GEP endocrine tumors are caused by overproduction of a specific hormone. For instance, the well-known Zollinger-Ellison syndrome is gastrin-mediated. The so-called 'insulinoma syndrome' depends on excessive production of insulin and proinsulin, resulting in hypoglycemia. The 'glucagonoma syndrome' is characterised by necrolytic migratory erythema, diabetes and diarrhoea. The Verner-Morrison syndrome, which is brought about by high circulating levels of vasointestinal peptide (VIP). produces severe secretory diarrhoea. Finally the 'somatostatinoma syndrome' involves gallbladder dysfunction and gallstones, diarrhoea with or without steatorrhea, and impaired glucose tolerance. The biochemical diagnosis of endocrine digestive tumors is based on general and specific markers. The best general markers are chromogranin A (CgA) and pancreatic polypeptide (PP). Specific markers for endocrine tumors include insulin, gastrin, glucagon, vaso intestinal polypeptide (VIP), somatostatin and the primary cathabolic product of serotonin, 5-hydroxyndoleacetic acid (5-HIAA). Localisation procedures commonly applied, in the diagnosis of endocrine tumours include ultrasound (US), computed tomography (CT) and somatostatin receptor scintigraphy (SRS).
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PMID:Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. 1176 60

Carcinoid tumors are very rare and originate mainly in the gastrointestinal tract. The tumor histology is ambiguous and malignancy is determined by metastases. Carcinoid tumors affect both sexes equally and have been found in all age groups. Many carcinoid tumors are found incidentally or from symptoms related to the hormones that the tumor produces. Carcinoid syndrome occurs when vast quantities of hormones are produced from GI carcinoid metastases or a non-GI primary tumor. The classic "carcinoid triad" associated with the syndrome includes flushing, diarrhea, and cardiac involvement. The hormone largely responsible for most of these symptoms is serotonin. Treatment consists of a wide-resection for local primaries and usually palliative, medical support for patients with metastases. The tumors are very slow-growing and patients have lived for up to 30 years after metastasis is diagnosed. Somatostatin analogue (lanreotide and octreotide) administration controls many of the carcinoid symptoms. Somatostatin is a naturally occurring gastrointestinal peptide (hormone) which can augment or counteract a wide variety of other peptides. This article provides an overview of carcinoid tumor and carcinoid syndrome including diagnosis and treatment. Aspects important to patient care will also be addressed.
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PMID:Carcinoid tumors and syndrome. 1205 78

Patients with clinically evident medullary thyroid cancer should have a total extracapsular thyroidectomy with bilateral central neck dissection and an ipsilateral prophylactic or therapeutic modified (functional) radical neck dissection when the primary tumor is greater than 1 cm and when the central neck nodes are positive. A prophylactic contralateral neck dissection should be done when the primary tumor is bilateral and when there is extensive lymphadenopathy on the side of the primary tumor. Patients who have gross, unresectable residual medullary thyroid cancer should receive postoperative external radiotherapy. Patients who are carriers of germ-line RET proto-oncogene point mutations or have an elevated (basal or stimulated) calcitonin levels on screening should have a prophylactic total thyroidectomy before age 6 years. In patients with an elevated basal or stimulated plasma calcitonin level and an intrathyroidal nodule on ultrasound, a total thyroidectomy and central neck lymph node dissection should be done. Patients with persistent or recurrent medullary thyroid cancer should have a complete thyroidectomy (if not done initially) and bilateral central and modified radical neck dissection, including upper mediastinal lymphadenectomy. Patients who are symptomatic from distant medullary thyroid cancer metastases (diarrhea, flushing, weight loss, or bone pain) should be treated with somatostatin analogs. Bone metastases should be resected if possible, and symptomatic lesions that are unresectable should be treated with external radiotherapy. Cytoreductive procedures such as radiofrequency ablation or cryoablation for liver metastases should be considered in symptomatic patients to reduce tumor burden. Localized pulmonary metastases should be resected. Chemotherapy or radioactive immunotherapy (iodine 131 labeled carcinoembryonic antigen monoclonal antibody) protocols should be considered in patients with nonoperative widely metastatic progressing medullary thyroid cancer.
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PMID:Medullary thyroid cancer. 1205 61

Carcinoids are rare endocrine tumors that can develop in several organs in the body. Clinically, patients can have a wide spectrum of signs and symptoms that range from incidental findings of a polyp during endoscopy to the carcinoid syndrome characterized by severe flushing, diarrhea, abdominal cramping, and life-threatening right-sided heart failure. Most carcinoid tumors are indolent but can metastasize to regional lymph nodes and to other organs, including the liver, bone, and the central nervous system. Treatment is determined by tumor location and by the presence of distant metastasis. Surgical resection of the tumor is advocated in patients with localized disease and can often be curative. Long-acting somatostatin analogs, including octreotide, octreotide long-acting repeatable, and lanreotide prolonged release, are effective in providing symptom relief in patients with the carcinoid syndrome. Patients with metastatic disease to the liver that is refractory to somatostatin treatment should be considered for hepatic artery occlusion. Overall, 5- and 10-year survival rates in patients with metastatic disease are favorable, although tumors can be resistant to most forms of medical or surgical therapy.
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PMID:Carcinoid tumors. 1207 68

Pancreatic carcinoids are very rare and usually have a poor prognosis. We describe a case of a pancreatic carcinoid with liver micrometastases in a female of 54 years of age in whom the tumor was without pronounced symptoms apart from rare episodes of flushing. The patient had been treated since November 1995 with the somatostatin analogue octreotide 200 micrograms twice daily for the first 2 years, with the long-acting analogue lanreotide 30 mg every 10 days for the following year, and then with octreotide LAR 20 mg every 28 days until the present. The flushing episodes disappeared completely, and the patient was well. Moreover, the dimensions of the tumor and the liver micrometastases remained stable during the observation period. As far as we known, this is the first case of a pancreatic carcinoid treated successfully with somatostatin analogues and having a satisfactory prognosis.
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PMID:Carcinoid of the pancreas. 1212 97

Carcinoids are neuroendocrine tumours of the gut which may also be found in the bronchus, pancreatic islets and retroperitoneum. They probably arise from gastrointestinal or bronchopulmonary pluripotential stem cells. Carcinoid tumours derived from these cells are potentially malignant; the strength of the tendency for aggressive growth correlates with the site of origin, depth of local penetration and the size of the tumour. Carcinoids occur sporadically or result from specific hereditary tumour syndromes. Mutations and/or aberrant expression of specific genes induce and promote tumour growth. Clinical features include local symptoms due to angulation or obstruction and hepatomegaly due to liver metastases. The carcinoid syndrome commonly involves flushing, diarrhoea, bronchospasm and hypotension. Other distinct syndromes may be caused by tumour release of products that may also be used as biochemical markers in diagnosis and follow-up. Scanning using radiolabelled octreotide, an analogue of somatostatin, sensitively identifies occult primary and metastatic deposits. Localized carcinoid tumours should be resected. Some patients benefit from hepatic resection. Palliation of symptoms is best achieved with octreotide. Hepatic artery chemoembolization may produce long-acting palliation. Further genetic characterization of the different types and stages of carcinoid development as well as assessment of gene expression profiles may improve differential diagnosis, prognosis and treatment.
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PMID:The spectrum of carcinoid tumours and carcinoid syndromes. 1462 99

The quantification of the tumour volume is essential for the assessment of therapy-induced changes. Traditional methods of assessing the response of neuroendocrine tumours using radiological methods yield poor results, particularly within the liver. The aim of this study was to establish whether it would be possible to identify a method using functional volumes to predict the response of tumours to various therapies. Twenty-two patients with neuroendocrine tumours of carcinoid type in the liver were treated with chemotherapy, chemo-embolization or 90Y-radiolabelled somatostatin analogues. All patients underwent 111In-pentetreotide single-photon emission computed tomography (SPECT) and computed tomography (CT) scan pre- and post-treatment. The tumour functional volume, a measure of metabolically active tumour tissue, was calculated from the SPECT images using a 10-point display; regions of interest were drawn around 50% of the maximum tumour activity, slice by slice, and then multiplied by the slice thickness (9.3 mm). Any difference in functional volume was compared with the CT response, using the Response Evaluation Criteria in Solid Tumours (RECIST), and clinical outcome. At 6 months after treatment, 14 patients showed a good clinical response, as measured by a reduction in pain, flushing or abdominal symptoms; the functional volume of the tumours in these patients decreased by a mean of 25% (range, 1-52%). Of the eight patients who showed no symptomatic relief, or in whom symptoms worsened, the functional volume increased by a mean of 74%. Using a change in functional volume of more than 25% as significant, SPECT predicted 13 of the 22 (59%) clinical outcomes correctly; if a 10% change was used, 18 of the 22 (81.1%) clinical outcomes were correctly predicted. However, CT, using RECIST, only predicted eight of the 22 (36%) clinical outcomes correctly. The assessment of the total functional volume by SPECT quantification is more useful than CT in monitoring tumour response after treatment, and the changes in functional volumes after therapy correlate well with the clinical response.
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PMID:Prediction of clinical outcome in treated neuroendocrine tumours of carcinoid type using functional volumes on 111In-pentetreotide SPECT imaging. 1509 43


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