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 tumors are neuroendocrine tumors with a very unpredictable clinical behavior. In the setting of hepatic metastases, the tumor's release of bioactive substances into the systemic circulation results in carcinoid syndrome: a constellation of symptoms among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most prominent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe fibrotic reaction which frequently involves the right-sided valves and may extend towards the subvalvular apparatus leading to valve thickening and retraction. Left-sided involvement is rare and mostly observed in the presence of an interatrial shunt, endobronchial tumor localization, and high tumor activity. Echocardiographic techniques often reveal noncoaptation of the valves, which are fixed in a semiopen position. In patients with advanced lesions and severe valvular dysfunction, surgery is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend towards improved surgical outcomes has triggered a more liberal referral for valve replacement. Carcinoid heart disease poses two distinct challenges for the anesthesiologist: carcinoid crisis and low cardiac output syndrome secondary to right ventricular failure. Carcinoid crisis, characterized by flushing, hypotension, and bronchospasm, may be precipitated by catecholamines and histamine releasing drugs used routinely in patients undergoing valve surgery. Although a broader utilization of octreotide have significantly simplified the anesthetic and perioperative management of these patients, a very balanced anesthetic technique is required to identify and manage low cardiac output syndrome.
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PMID:Current concepts in diagnosis and perioperative management of carcinoid heart disease. 2317 18

Supportive care of patients with functional neuroendocrine tumors (NETs) has evolved to include the use of multiple targeted agents to control paraneoplastic states and newer surgical and interventional radiologic techniques to reduce tumor bulk. Challenges encountered by the clinician are the recognition of specific symptom complexes, selecting the relevant laboratory tests and radiologic/scintigraphic scans, and the timing of intervention(s). Individual variables such as the severity of symptoms in the context of primary and metastatic disease sites, tumor bulk, comorbidities, and previous treatment are factors determining the prioritization of specific treatment regimens for patients with functional NETs. Symptoms such as flushing, secretory diarrhea, hypercalcemia, hyper /hypoglycemia, hypercortisolism, and peptic ulcers should improve with decreasing the elevated amino acid and/or peptide levels produced by NETs. These paraneoplastic symptoms may be accompanied by complaints related to tumor burden such as fatigue, pain, early satiety, anorexia, weight loss, night sweats, and/or symptoms secondary to adverse drug effects such as mucositis, dysgeusia, diarrhea, rash, hypertension, and myelosuppression. Developing a comprehensive continuum of care plan early in disease management assists in controlling the presenting signs and symptoms, and in minimizing disease- and/or treatment-related side effects. This guide serves as a framework to manage the signs and symptoms of metastatic functional neuroendocrine tumors.
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PMID:Practical guide to supportive care of patients with functional neuroendocrine tumors. 2339 Nov 12

Carcinoid tumors are neuroendocrine tumors with an unpredictable clinical behavior. In the setting of hepatic metastases, the release of bioactive amines from the tumor into the systemic circulation results in carcinoid syndrome: a constellation of clinical symptoms, among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most frequent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe endocardial fibrotic reaction that leads to progressive valve thickening and retraction. Imaging studies commonly reveal severe right-sided valve disease, with fixed leaflets or cusps in a semiopen position. The replacement of the right-sided valves, including the patch enlargement of the right ventricular outflow tract, is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend toward improved surgical outcomes has triggered a more liberal referral for valve replacement during the past decade.
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PMID:Surgical management of carcinoid heart valve disease. 2346 73

The prevalence of intestinal neuroendocrine tumours, also known as carcinoid tumours, has increased significantly over the past three decades. Tumours of the distal small intestine (midgut) are often indolent, but are characterized by a high potential to metastasize to the small-bowel mesentery and liver. Patients with distant metastases are prone to development of the carcinoid syndrome, a constellation of symptoms which includes flushing, diarrhoea, and valvular heart disease. The carcinoid syndrome is caused by secretion of serotonin and other vasoactive substances into the systemic circulation. Treatment options for metastatic intestinal NETs have expanded in recent years. Of particular importance has been the development of somatostatin-analogue therapies. Somatostatin analogues were originally introduced for palliation of the carcinoid syndrome; however recent clinical trials have demonstrated that they can exert an inhibitory effect on tumour growth. Other novel agents targeting the VEGF and mTOR pathways have recently been evaluated in phase III trials, however their role in the management of small-intestinal NETs remains controversial. This article examines the biological characteristics of small intestinal NETs, summarizes current guidelines on classification, staging and grading, and reviews developments in locoregional and systemic therapy.
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PMID:Neuroendocrine tumours of the small intestine. 2358 17

Carcinoid tumor is a slow-growing type of neuroendocrine tumor, originating in the enterochromaffin cells and secreting mainly serotonin. The diagnosis is based on clinical symptoms, hormone levels, radiological and nuclear imaging, and histological confirmation. The clinical symptoms are characterized by flushing, diarrhea, abdominal pain, telangiectasia and/or bronchoconstriction. However, most patients have metastatic disease at diagnosis because the clinic goes unnoticed or are ascribed to other abdominal conditions. We report the clinical symptoms, hormone levels, radiological and nuclear imaging, histological diagnosis, treatment and evaluation of a 44-year-old female patient with congestive heart failure secondary to carcinoid heart disease in the context of liver metastases of an ileum carcinoid tumor.
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PMID:Metastatic carcinoid tumor presenting as right sided heart failure. 2382 84

Recurrent, pulseless-electrical-activity (PEA) cardiac arrests were the novel presentation of untreated systemic mastocytosis in an 85-year-old woman who lacked cutaneous findings of mastocytosis. Despite prior implantation of a dual-chamber cardiac pacemaker 3 weeks previously for similar spells, she experienced a PEA arrest accompanied by flushing, increased urinary N-methylhistamine excretion and serum tryptase values on the day of presentation to our clinic. Bone marrow biopsy findings conducted to rule out breast cancer metastases showed 30% mast cell infiltration, aberrant expression of CD25 and a positive c-kit Asp816Val mutation. Treatment with a combination of H1 and H2 receptor blockers reduced flushing and eliminated hypotension. Maintenance medication included aspirin, cetirizine, ranitidine, montelukast, oral cromolyn sodium and an epinephrine autoinjector (as needed). At 6-month follow-up, the patient remained free of PEA arrests, flushing, or any clinical signs of mastocytosis or mast cell degranulation. PEA cardiac arrests may therefore be a presenting sign of untreated systemic mastocytosis.
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PMID:Presentation of untreated systemic mastocytosis as recurrent, pulseless-electrical-activity cardiac arrests resistant to cardiac pacemaker. 2433 43

Midgut neuroendocrine tumors (MNETs) are rare, and the primary tumor is usually small and difficult to visualize at imaging. Patients often present late with extensive liver and nodal metastases and may experience symptoms secondary to the release of active substances by the primary tumor, such as serotonin and its metabolites, which have local and systemic effects. Locally, this causes desmoplasia and vascular encasement and may lead to small bowel obstruction and ischemia, with significant morbidity and mortality. Systemically, the release of active substances into the circulation can cause flushing, diarrhea, and abdominal pain (carcinoid syndrome); these substances can be detected in urine and blood serum and used as markers for diagnosis and treatment follow-up. MNETs retain expression of specific peptide receptors such as somatostatin receptors, which will bind to synthetic somatostatin analogs such as octreotide. This feature is useful for functional imaging of patients with MNETs and for peptide receptor radionuclide therapy using somatostatin analogs. Resection of the primary tumor is advocated, even in patients with extensive metastases, because it may prevent development of local complications, can help control systemic symptoms, and has been shown to confer some survival advantage. Computed tomography and functional imaging are used to identify the primary tumor and assess its resectability. The main factors governing resectability are patient comorbidities (eg, carcinoid heart disease), vascular involvement, and desmoplasia.
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PMID:Midgut neuroendocrine tumors: imaging assessment for surgical resection. 2461 88

The patient, C.P., is a 59-year-old woman who was diagnosed with metastatic carcinoid of the terminal ileum in May 2003. In June 2003, she underwent an extensive resection including hemicolectomy, cholecystectomy, distal pancreatectomy, and splenectomy with metastatic disease in her pancreas, mesentery, and liver. She had been treated with octreotide, everolimus, oxaliplatin, and multiple hepatic artery embolizations in the past eight years and, most recently, capecitabine and bevacizumab with monthly octreotide. She has had intermittent pleural effusions not requiring intervention and a trace pericardial effusion. Her tumor is functional, meaning it demonstrates hormonal hypersecretion which causes flushing, diarrhea, bronchospasm, and abdominal pain.
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PMID:Carcinoid heart disease. 2535 25

Medullary thyroid cancer is a rare neuroendocrine neoplasm that arises from the parafollicular C cells that produce calcitonin, a hormone essential for the regulation of calcium metabolism. It accounts for 4%-10% of all thyroid cancers. In most cases (75%-80%), it is sporadic, while in other cases it is part of the multiple endocrine neoplasia (MEN) syndrome. Most often, medullary thyroid carcinoma is presenting as a solitary nodule. At the time of diagnosis, about half of the patients have enlarged cervical lymph nodes, while a small number of patients have distant metastases in the liver, lungs, bones and brain. If the tumor is hormone active, the patient may have systemic symptoms such as diarrhea or flushing. Ultrasonically, medullary carcinoma usually appears as a hypoechogenic node with marked vascularity and uneven contours that can sometimes contain microcalcifications, and in most cases is located in the upper poles of the thyroid. The sample obtained by fine needle aspiration is usually cellular, tumor cells are disseminated or arranged in poorly cohesive groups. They are most often plasmacytoid, but sometimes can also be spindled. The nuclei are eccentric and chromatin shows features of neuroendocrine tumors. Cytoplasms of tumor cells are abundant, triangular or polygonal, amphophilic, finely granulated and unsharply limited. Background is clean and sometimes amyloid can be found. Depending on the cytologic picture, differential diagnostic problems can be well differentiated thyroid tumors, primarily follicular neoplasm, lymphomas, poorly differentiated insular carcinoma, metastatic small cell carcinoma, mesenchymal tumors or melanoma. In case of differential diagnostic difficulties, of great help is to determine calcitonin immunocytochemically or in aspirate or serum. Medullary carcinoma may show low progression and long-time survival, but can also be a rapidly progressive tumor where survival is measured in months. Good prognostic indicators are younger age, female gender, tumor size, tumor localization only in the thyroid gland, and absence of local or distant metastases. In conclusion, medullary thyroid carcinoma is a rare tumor in which we still have to think in diagnosing thyroid nodules, mainly due to adequate preoperative preparation in terms of exclusion of pheochromocytoma in MEN syndrome. Cytologic picture in some cases is typical; however, immunocytochemical evidence of calcitonin is of great help to confirm morphological diagnosis.
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PMID:[PREOPERATIVE DIAGNOSTICS OF THYROID MEDULLARY CARCINOMA WITH EMPHASIS ON CYTOMORPHOLOGICAL FEATURES AND DIFFERENTIAL DIAGNOSIS OF PRIMARY AND SECONDARY THYROID TUMORS]. 2628 71

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare, generally indolent neoplasms that can arise throughout the gastrointestinal system. Some GEP-NETs, known as functional, secrete hormones that can lead to a complex of symptoms. Classical carcinoid syndrome is associated with flushing, diarrhea, bronchospasm, and symptoms of valvular heart disease. GEP-NETs are classified according to the primary tumor site, functionality of the disease, and histology. Treatment is guided by the resectability of the tumor, the location and extent of metastases, and the presence of clinical symptoms. Typically, first-line treatment of patients with unresectable disease includes the use of somatostatin analogs, such as octreotide LAR depot or lanreotide depot/autogel, which was recently approved by the US Food and Drug Administration for treatment of GEP-NETs. Somatostatin analogs can improve the severe diarrhea/flushing episodes that may be associated with metastatic carcinoid tumors. For patients with pancreatic NETs, additional approved treatment options include the targeted agents everolimus and sunitinib, which have demonstrated antitumor activity. Chemotherapy may also have a selective role, particularly in pancreatic NETs. Localized approaches, including cytoreductive surgery, hepatic arterial embolization, and ablative therapies, may be used for palliative treatment in patients with liver metastases.
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PMID:New and Emerging Treatment Options for Gastroenteropancreatic Neuroendocrine Tumors. 2643 Sep 56


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