Carcinoid syndrome: update on the pathophysiology and treatment

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Term Occurence Count Dictionary
niacin 4 endocrinologydiseasesdrugs
pellagra 7 endocrinologydiseases
telotristat ethyl 4 endocrinologydiseasesdrugs
Octreotide 3 endocrinologydiseasesdrugs
cabergoline 1 endocrinologydiseasesdrugs
carcinoid 27 endocrinologydiseases
everolimus 2 endocrinologydiseasesdrugs
neuroendocrine tumor 4 endocrinologydiseases

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Select Drug Character Offset Drug Term Instance
Octreotide 22134 Approximately 80% of well-differentiated tumors express somatostatin receptors in the NET cell surface [60]. Octreotide and lanreotide bind to somatostatin receptors and inhibit the secretion of several hormones and vasoactive
Octreotide 22345 vasoactive substances, thus improving flushing and diarrhea symptoms in over 80% patients with CS [61]. Octreotide in its depot form is called Octreotide LAR and can be administered intramuscularly at a dose of 20 to
Octreotide 22384 flushing and diarrhea symptoms in over 80% patients with CS [61]. Octreotide in its depot form is called Octreotide LAR and can be administered intramuscularly at a dose of 20 to 30 mg every 4 weeks. Lanreotide is administered
cabergoline 15400 to an important role of 5-HT in the development of CHD: serotonergic drugs (fenfluramine, pergolide, cabergoline , and ergotamine by-products) have been shown to cause valve fibrosis similar to that observed with CHD
everolimus 25897 the toxicity profile is unfavorable.Retrospective studies suggest symptomatic benefit with the use of everolimus in patients with CS [72]. Additionally, the phase III placebo-controlled trial RADIANT 2 [73] demonstrated
everolimus 26020 with CS [72]. Additionally, the phase III placebo-controlled trial RADIANT 2 [73] demonstrated that everolimus combined with octreotide led to more reduction in the levels of urinary 5-HIAA in comparison with the
niacin 10594 that produce either 5-HT or beta-nicotinamide adenine dinucleotide (NAD), which is an active form of niacin (vitamin B3). Since tumors associated with CS can consume up to 60% of tryptophan in the human body
niacin 10804 [25], if not properly treated, patients may experience adverse events resulting from tryptophan and/or niacin deficiencies. Such a state of deprivation may cause several problems, including pellagra and neurocognitive
niacin 13174 neurocognitive dysfunction occurs in patients with CS.PellagraPellagra is a clinical condition caused by niacin deficiency (vitamin B3) characterized by dermatitis, diarrhea and dementia in severe cases. The actual
niacin 13930 including intense skin pruritus resulting from pellagra. Figure 2 demonstrates a patient with pellagra and niacin deficiency due to long-term CS that was not adequately controlled due to lack of adherence to somatostatin
telotristat ethyl 8077 bowel movements and urinary 5-HIAA levels demonstrated in the phase III placebo-controlled trial of telotristat ethyl , an oral inhibitor of 5-HT synthesis [17]. Nevertheless, other substances may be cosecreted with 5-HT,
telotristat ethyl 12139 development of this drug was halted. Recently, in the phase III placebo-controlled trial TELESTAR [30] with telotristat ethyl , patients who received 500 mg of telotristat three times per day (TID) reported more depressive symptoms
telotristat ethyl 12434 placebo) than those who were treated with 250 mg TID or placebo. Longer duration of treatment with telotristat ethyl and more studies with a larger number of patients are necessary to properly evaluate the neurological
telotristat ethyl 29221 functional class [89]. More studies are needed to replicate these encouraging results with bosentan.Because telotristat ethyl reduces the levels of 5-HIAA in 24-h urine tests, it is may be a promising treatment to prevent or delay
Select Disease Character Offset Disease Term Instance
carcinoid 305 /2018AbstractApproximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors.
carcinoid 745 work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents
carcinoid 890 somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed.
carcinoid 1084 interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications,
carcinoid 1168 potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include
carcinoid 1213 their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary
carcinoid 1517 and tachykinins.Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology,
carcinoid 2190 telangiectasia, dyspnea and ultimately fibrotic complications such as mesenteric and retroperitoneal fibroses and carcinoid heart disease (CHD) [2]. CS is predominantly associated with neuroendocrine tumors (NETs) that arise
carcinoid 2408 the midgut in the setting of extensive liver metastases but may be present in patients with bronchial carcinoid s and, more rarely, in patients with pancreatic NETs. In patients with extensive liver metastases, a
carcinoid 2665 not completely metabolized by hepatic or pulmonary cells and enter the systemic circulation, causing carcinoid symptoms; carcinoid-produced substances may also enter the systemic circulation when the patient exhibits
carcinoid 2685 metabolized by hepatic or pulmonary cells and enter the systemic circulation, causing carcinoid symptoms; carcinoid -produced substances may also enter the systemic circulation when the patient exhibits forame ovale or
carcinoid 4511 as being potentially related to CS; however, their individual contributions in triggering different carcinoid symptoms and complications, such as CHD, remain unclear [7]. These substances include serotonin (5-HT),
carcinoid 4822 kallikrein, prostaglandins, and tachykinins [2]. Below, we discuss the characteristics of the most common carcinoid symptoms and their related complications, which are summarized in Table 1 and Figure 1.FlushingFlushing
carcinoid 5496 prostaglandins, provoke flushing when they are not inactivated by hepatocytes [10],[11]. The release of carcinoid -related substances is usually triggered by amine-rich foods (chocolate, banana, kiwi, avocado and nuts),
carcinoid 5861 serotoninergic antidepressants was safe to treat depressive symptoms and did not significantly worsen carcinoid symptoms [12].While 5-HT is the proposed main causative agent of CS-associated flushing, as evidenced
carcinoid 8891 histamine and 5-HT by the tumor are probably linked to the mechanisms of bronchospasm.Carcinoid crisisA carcinoid crisis is a serious and potentially life-threatening exacerbation of CS due to the release of large
carcinoid 9420 prophylactic use of short-acting somatostatin analogues has been suggested and widely used to prevent carcinoid crisis that could be induced by invasive procedures, some patients still experience uncontrolled carcinoid
carcinoid 9527 carcinoid crisis that could be induced by invasive procedures, some patients still experience uncontrolled carcinoid symptoms [2],[21]. Recently, Massimino et al. [22] reported that a 500-μg bolus of octreotide acetate
carcinoid 9711 bolus of octreotide acetate administered preoperatively was insufficient to prevent intraoperative carcinoid crisis. In contrast, Woltering et al. [23] observed that only 3.4% of CS patients experienced intraoperative
carcinoid 9947 continuous infusion of IV octreotide acetate preoperatively. Given the life-threatening consequences of carcinoid crisis, we recommend that prophylactic and intraoperative octreotide be administered to all patients
carcinoid 20349 ability to stimulate collagen synthesis [58].DiagnosisThe diagnosis of CS requires the combination of carcinoid symptoms and evidence of elevated levels of 5-HIAA in a 24-h urine sample. The sensitivity and specificity
carcinoid 23341 difficult to compare results from different studies. Most studies consider refractory CS as uncontrolled carcinoid symptoms despite label doses of somatostatin analogues [64]. However, “uncontrolled” is a subjective
carcinoid 26830 Another effective therapy for refractory CS is liver transarterial embolization, which can control carcinoid symptoms in up to 75% of patients [80]-[82]. Importantly, short-acting octreotide should be administered
carcinoid 27019 octreotide should be administered before, during and after the procedure in all these situations to avoid a carcinoid crisis [24].Another promising strategy is the use of somatostatin analogue radiolabeled peptide therapy
carcinoid 30767 for CS and have excellent efficacy. Unfortunately, the disease progresses, and patients experience carcinoid symptoms that are refractory to label doses to somatostatin analogue treatment. In this scenario, locoregional
carcinoid 31761 15% of the manuscript, guided the article and reviewed it.Figure 1Summary of the pathophysiology of carcinoid syndrome.Figure 2Patient with pellagra, in whom we observed dry skin and scratches from intense itching.Figure
carcinoid 31969 itching.Figure 3CT showing a characteristic image of mesenteric fibrosis.Table 1Symptoms and signs of carcinoid syndrome.SymptomFrequency %CharacteristicsInvolved mediatorsFlushing90 [9]Foregut: long-lasting, purple
neuroendocrine tumor 270 8/2018Publication date (ppub): /2018AbstractApproximately 30-40% of patients with well-differentiated neuroendocrine tumor s present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of
neuroendocrine tumor 559 negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumor s; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life.
neuroendocrine tumor 719 lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumor s and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While
neuroendocrine tumor 2261 retroperitoneal fibroses and carcinoid heart disease (CHD) [2]. CS is predominantly associated with neuroendocrine tumor s (NETs) that arise from the midgut in the setting of extensive liver metastases but may be present in
pellagra 10891 tryptophan and/or niacin deficiencies. Such a state of deprivation may cause several problems, including pellagra and neurocognitive disturbances, as discussed below.Neuropsychological symptomsIt has been estimated
pellagra 13298 (vitamin B3) characterized by dermatitis, diarrhea and dementia in severe cases. The actual prevalence of pellagra in patients with CS is unknown, although some studies have reported that approximately 5% of CS patients
pellagra 13423 CS is unknown, although some studies have reported that approximately 5% of CS patients experience pellagra [31]. Fortunately, pellagra is rarely seen after the incorporation of somatostatin analogues into the
pellagra 13451 some studies have reported that approximately 5% of CS patients experience pellagra [31]. Fortunately, pellagra is rarely seen after the incorporation of somatostatin analogues into the therapeutic arsenal for NET
pellagra 13870 patients presenting with long-term uncontrolled symptoms, including intense skin pruritus resulting from pellagra . Figure 2 demonstrates a patient with pellagra and niacin deficiency due to long-term CS that was not
pellagra 13917 symptoms, including intense skin pruritus resulting from pellagra. Figure 2 demonstrates a patient with pellagra and niacin deficiency due to long-term CS that was not adequately controlled due to lack of adherence
pellagra 31801 article and reviewed it.Figure 1Summary of the pathophysiology of carcinoid syndrome.Figure 2Patient with pellagra , in whom we observed dry skin and scratches from intense itching.Figure 3CT showing a characteristic

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