2,455 results match your criteria Malignant Carcinoid Syndrome

Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated with Atypical Thymic Carcinoid: A Case Report.

Ann Thorac Cardiovasc Surg 2021 Apr 27. Epub 2021 Apr 27.

Division of Thoracic Surgery, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease, occasionally accompanied by malignant tumors. Immunosuppressive therapy is the mainstay treatment for idiopathic NMOSD; no guidelines have been published for paraneoplastic NMOSD because it is rarely reported in the literature. We report a rare case of a 67-year-old man with paraneoplastic NMOSD associated with thymic carcinoid whose cells expressed aquaporin-4 antibody. Read More

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Value of a patient-reported-outcome measure of carcinoid syndrome symptoms.

Eur J Endocrinol 2021 May;184(5):711-722

Service d'Oncologie Médicale et Hépatogastroentérologie, Hospices Civil de Lyon, Lyon, France.

Objective: Literature on patient-reported outcomes (PRO) of carcinoid syndrome symptoms (CSS) is scarce. We used a patient-reported outcome measure (PROM) to evaluate CSS, the domains of daily life impacted by CSS, the main symptoms that affect daily life, its change according to clinical, biological and morphological evolution, and the risk factors for a poor PRO-CSS score.

Methods: Patients completed the PRO-CSS, EORTC-QLQ30, and GI-NET21 questionnaires at the time of their clinical, laboratory, and morphological assessments in a multicentre French cohort study from February 2019 to May 2020. Read More

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Update in carcinoid heart disease - the heart of the matter.

Rev Endocr Metab Disord 2021 Jan 14. Epub 2021 Jan 14.

Neuroendocrine Tumor Unit, Endocrinology and Metabolism Department, Division of Medicine, Hadassah-Hebrew University Medical Center, P.O.B. 12000, 91120, Jerusalem, Israel.

Carcinoid heart disease (CHD) is a paraneoplastic cardiac manifestation occurring in patients with carcinoid syndrome (CS) and advanced neuroendocrine malignancy. In about 20-40% of patients with CS, chronic exposure to tumor-released circulating vasoactive peptides typically results in right-sided valvular fibrosis leading to valve dysfunction and right heart failure. CHD remains a significant cause of morbidity and mortality. Read More

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January 2021

A clinical and echocardiographic case report of carcinoid-related valvular heart disease.

Eur Heart J Case Rep 2020 Dec 6;4(6):1-4. Epub 2020 Nov 6.

Division of Cardiology, University of Alabama Birmingham, Birmingham, AL, USA.

Background: Carcinoid syndrome is a rare disease caused by malignant neuroendocrine neoplasms. When vasoactive substances enter the systemic circulation, the triad of cutaneous flushing, bronchospasm, and diarrhoea often characterize carcinoid syndrome. Rarely, carcinoid syndrome can progress to involve the cardiac system, a condition known as carcinoid heart disease, often affecting right-sided valvular structures. Read More

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December 2020

Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.

Expert Opin Pharmacother 2021 Apr 11;22(6):685-693. Epub 2020 Nov 11.

Digestive Diseases Branch, NIDDK,NIH, Bethesda, MD, USA.

: In the past, controlling the hormone-excess-state was the main determinant of survival in Functional-Neuroendocrine-Neoplasm-syndromes (F-NENs). This was difficult because the pharmacological-armamentarium available was limited. Recently, new therapeutic strategies have increased but it also generated controversies/uncertainties. Read More

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Surgical aspects of valve replacement in carcinoid heart disease.

J Card Surg 2021 Jan 30;36(1):290-294. Epub 2020 Oct 30.

Department of Cardiothoracic Surgery and Anesthesiology, University Hospital, Uppsala, Sweden.

Tricuspid and pulmonary valve replacement in patients with advanced carcinoid heart disease (CaHD) reduces right heart failure and improves prognosis. The surgical literature is limited concerning description of technical aspects of valve replacement in CaHD. Although a dedicated multidisciplinary care is required for these frail patients, optimization of surgical technique is important and may lead to better postoperative outcomes. Read More

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January 2021

A novel mutation of the MEN1 gene in a patient with multiple endocrine neoplasia type 1 and recurrent fibromyxoid sarcoma - a case report.

BMC Med Genet 2020 09 29;21(1):190. Epub 2020 Sep 29.

Department of Endocrinology and Diabetology, University Hospital Centre Split, Soltanska 1, Split, Croatia.

Background: Multiple endocrine neoplasia type 1 (MEN1) syndrome is usually accompanied by endocrine tumors, but non-endocrine tumors can occur as well. However, the coexistence of MEN1 syndrome and malignant tumor such as low-grade fibromyxoid sarcoma has not been described in the literature. Moreover, the MEN1 gene mutations have not been identified in patients with fibromyxoid sarcoma, so far. Read More

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September 2020

Antiproliferative Effects of Telotristat Ethyl in Patients with Neuroendocrine Tumors: The TELEACE Real-World Chart Review Study.

Cancer Manag Res 2020 30;12:6607-6614. Epub 2020 Jul 30.

Neuroendocrine Tumor Program at Penn Medicine, Philadelphia, PA, USA.

Purpose: Neuroendocrine tumors (NETs) associated with carcinoid syndrome (CS) overproduce serotonin, mediated by tryptophan hydroxylase-1 (TPH1). The TPH inhibitor telotristat ethyl (TE) reduces peripheral serotonin and relieves CS symptoms. We conducted a real-world clinical practice study to explore the effects of TE on tumor growth in patients with NETs and CS. Read More

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[Carcinoid heart disease].

Rev Med Liege 2020 Jul;75(7-8):478-483

Service de Chirurgie cardiovasculaire et thoracique, CHU Liège, Belgique.

Carcinoid syndrome is the term applied to a constellation of symptoms mediated by a variety of humoral factors produced by the well-differentiated neuroendocrine tumours (NET) localised usually in the gastrointestinal tract and the lungs. This syndrome includes a characteristic triad of diarrhea, flushing and cardiac disease. This cardiopathy occurs in 20 % of the cases and prevails on the right side of the heart. Read More

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Assessment of serotonin concentration in patients with a small-intestine neuroendocrine neoplasm and carcinoid syndrome treated with somatostatin analogues.

Pol Arch Intern Med 2020 10 9;130(10):903-905. Epub 2020 Jul 9.

Department of Endocrinology, Metabolism and Internal Diseases, Poznan University of Medical Sciences, Poznań, Poland

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October 2020

Cardiac metastasis of neuroendocrine tumor of the thymus diagnosed by cardiac magnetic resonance T1 mapping: a case report.

J Int Med Res 2020 Jun;48(6):300060520931678

Department of Radiology, The Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, China.

Carcinoid heart disease is a late complication of carcinoid syndrome, and imaging can help in the diagnosis of diffuse cardiac metastasis. We herein report a case of diffuse cardiac metastasis of a neuroendocrine tumor of the thymus diagnosed using different imaging modalities. Cardiac magnetic resonance imaging played an important role in the diagnosis. Read More

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Regarding "TELEPRO: Patient-Reported Carcinoid Syndrome Symptom Improvement Following Initiation of Telotristat Ethyl in the Real World".

Oncologist 2020 07 28;25(7):e1132. Epub 2020 May 28.

Department of Gastroenterology, King's Health Partners Neuroendocrine Tumor Centre, Institute of Liver Studies, and European Neuroendocrine Tumor Society (ENETS) Centre of Excellence, King's College Hospital, London, United Kingdom.

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Timing of peptide receptor radiotargeted therapy in relation to cardiac valve surgery for carcinoid heart disease in patients with neuroendocrine metastases and cardiac syndrome. A single-centre study from a centre of excellence.

Nucl Med Commun 2020 Jun;41(6):575-581

Kings College Hospital Nuclear Medicine Department.

Introduction: Perioperative mortality of patients who undergo heart valve surgery for carcinoid heart valve disease has been observed to be high (5%-10%). We investigated whether peptide receptor radiotherapy with lutetium-177 dotatate can be used safely in patients with neuroendocrine neoplasm carcinoid heart valve disease and if there is associated survival advantage by reducing overall exposure of the valves to high doses of vasoactive peptides.

Method: Retrospective case notes review was performed on 18 neuroendocrine neoplasm patients (mean 60 years), who underwent heart valve surgery between 2003 and 2017 for carcinoid heart valve disease, 9 of whom received peptide receptor radiotherapy in addition to surgery. Read More

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Evaluation of 5-hydroxyindoloacetic acid excretion in urine in patients with small intestine neuroendocrine neoplasm and carcinoid syndrome treated with somatostatin analogues.

Neuro Endocrinol Lett 2019 Dec;40(7-8):315-318

Poznan University of Medical Sciences, Department of Endocrinology, Metabolism and Internal Medicine, Przybyszewskiego 49, 60-355 Poznan, Poland.

Background: The assessment of hormonal function of neuroendocrine neoplasm (NEN) is an important stage in the diagnosis and monitoring of these diseases treatment. Objective of this study was to analyze the results of urinary excretion of 5-hydroxyindoloacetic acid (5-HIAA) in patients with carcinoid syndrome treated with somatostatin analogues, depending on the histologic maturity, degree of liver involvement and stage of the disease.

Methods: The final group comprised of 41 patients. Read More

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December 2019

Multiple malignant transformations of an ovarian mature cystic teratoma.

Ecancermedicalscience 2020 4;14:1009. Epub 2020 Feb 4.

Department of Gynecologic Oncology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY 10021, USA.

Background: Malignant transformation of mature cystic teratomas (MCTs) is a rare phenomenon. The most common histology of a malignant transformation is squamous cell carcinoma, and there are limited reports of multiple malignancies arising in a single MCT. Further data are necessary to guide management of these atypical cases. Read More

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February 2020

A Budget Impact Model of the Addition of Telotristat Ethyl Treatment to the Standard of Care in Patients with Uncontrolled Carcinoid Syndrome.

Pharmacoeconomics 2020 06;38(6):607-618

Ipsen Pharma, 65 Quai Georges Gorse, 92100, Boulogne-Billancourt, France.

Background: Carcinoid syndrome, a rare condition in patients with neuroendocrine tumours, characterised by flushing and diarrhoea, severely affects patients' quality of life. The current carcinoid syndrome standard of care includes somatostatin analogues, but some patients experience uncontrolled symptoms despite somatostatin analogue therapy. Telotristat ethyl is a novel treatment approved by the European Medicines Agency (EMA) and US FDA that significantly reduces bowel movement frequency in patients with uncontrolled carcinoid syndrome. Read More

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Time to Sustained Improvement in Bowel Movement Frequency with Telotristat Ethyl: Analyses of Phase III Studies in Carcinoid Syndrome.

J Gastrointest Cancer 2021 Mar;52(1):212-221

Department of Hepatology and Gastroenterology, Charité-Universitätsmedizin, Berlin, Germany.

Background: Telotristat ethyl is approved to treat carcinoid syndrome diarrhea in combination with somatostatin analogs. In TELESTAR and TELECAST phase III studies, patients with carcinoid syndrome received telotristat ethyl 250 or 500 mg 3 times per day (tid) or placebo tid in addition to somatostatin analogs. The aim of this prespecified analysis was to examine the time to reductions in bowel movements (BMs) in the TELESTAR and TELECAST studies using survival analysis methods. Read More

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Endoscopic Resection of a Pediatric Carcinoid Lung Tumor Presenting as Persistent Pneumonia.

S D Med 2020 Feb;73(2):54-58

Department of Pediatrics, Avera McKennan Children's Hospital and University Health Center.

Primary lung tumors are very rare in children and constitute only 0.2 percent of all pediatric malignancies. Carcinoids are the most common primary pediatric lung tumor and account for 80 percent of all primary malignant bronchial tumors. Read More

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February 2020

Clinical Benefits of Telotristat Ethyl in Patients With Neuroendocrine Tumors and Low Bowel Movement Frequency: An Observational Patient-Reported Outcomes Study.

Pancreas 2020 03;49(3):408-412

Medical Affairs, Lexicon Pharmaceuticals, Inc, The Woodlands, TX.

Objectives: We evaluated carcinoid syndrome (CS) symptoms and the real-world effectiveness of telotristat ethyl (TE) among patients with ≤3 bowel movements (BM) per day.

Methods: Patients with CS initiating TE between March and November 2017 could participate in a nurse support program collecting demographic and CS symptom data before TE initiation (baseline) and during ≥1 monthly follow-up within 3 months. Symptoms for patients averaging ≤3 BM/d at baseline were evaluated using pre/post-Student t tests. Read More

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Flushing as atypical initial presentation of functional gallbladder neuroendocrine carcinoma: A case report.

World J Gastroenterol 2020 Feb;26(6):686-695

Department of General Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China.

Background: Neuroendocrine neoplasms are rarely located in the gallbladder (GB), and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.

Case Summary: We report a case of GB neuroendocrine carcinoma (GB-NEC) in a 65-year-old man, who presented with flushing for 2 mo. Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis. Read More

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February 2020

CSTI-300 (SMP-100); a Novel 5-HT Receptor Partial Agonist with Potential to Treat Patients with Irritable Bowel Syndrome or Carcinoid Syndrome.

J Pharmacol Exp Ther 2020 04 26;373(1):122-134. Epub 2020 Feb 26.

Neuropharmacology Research Group, Institute of Clinical Sciences (A.R., G.G., A.J.C., C.A.B., O.Q., N.M.B.) and Institute of Immunology and Immunotherapy (Z.S.), College of Medical and Dental Sciences and DB - Diagnostics, Drugs, Devices and Biomarkers, Cancer Research UK Clinical Trials Unit (K.B.), University of Birmingham, Edgbaston, Birmingham, United Kingdom; Department of Life Science, School of Health Sciences, Birmingham City University, Birmingham, United Kingdom (A.D.P.); Shanghai Medicilon Inc., Shanghai, China (C.C.); Chengdu SciMount Pharmatech Co. Ltd., Chengdu, China (D.X., J.H.); ConSynance Therapeutics, Inc., Rensselaer, New York (S.L., P.R.G.); Albany Molecular Research, Inc., Albany, New York (D.D.M., N.A.M.); and Sargent Consulting, Hendersonville, North Carolina (B.J.S.)

The 5-hydroxytryptamine (5-HT) (serotonin) 5-HT receptor represents a clinical target for antagonists to deliver symptomatic relief to patients with diarrhea-predominant irritable bowel syndrome (IBS-d) or carcinoid syndrome. Unfortunately, this pharmacological strategy can present side effects (e.g. Read More

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Must antidepressants be avoided in patients with neuroendocrine tumors? Results of a systematic review.

Palliat Support Care 2020 10;18(5):602-608

Department of Psychiatry and Behavioral Sciences, Memorial Sloan Kettering Cancer Center, New York, NY.

Objective: Symptoms of depression and anxiety are common in neuroendocrine tumor (NET), yet controversy exists over whether serotonin-mediated antidepressants (SAs) are safe in this population. We sought to address this knowledge gap.

Method: Following PRISMA guidelines, we conducted a systematic review to identify NET patients who were prescribed SA. Read More

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October 2020

Anesthesia for Endocrine Emergencies.

Anesthesiol Clin 2020 Mar 2;38(1):149-163. Epub 2020 Jan 2.

Division of Cardiothoracic Anesthesiology, Department of Anesthesiology, Virginia Mason Medical Center, 1100 Ninth Avenue, B2-AN, Seattle, WA 98101, USA; Division of Critical Care Medicine, Virginia Mason Medical Center, 1100 Ninth Avenue, B2-AN, Seattle, WA 98101, USA. Electronic address:

Although endocrine emergencies are not common occurrences, their identification and careful perioperative management are of paramount importance for reduction of patient morbidity and mortality. The most common critical endocrine abnormalities are associated with functional tumors, such as pheochromocytomas, insulinomas, and carcinoid tumors, leading to carcinoid syndrome, abnormal thyroid function, or disturbances in the hypothalamus-pituitary-adrenal axis, causing adrenal insufficiency. This article aims to discuss the pathophysiology, diagnosis, and perioperative management of pheochromocytomas, hyperthyroidism, hypothyroidism, adrenal insufficiency, carcinoid disease, and insulinomas. Read More

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Direct costs of carcinoid syndrome diarrhea among adults in the United States.

World J Gastroenterol 2019 Dec;25(47):6857-6865

Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.

Background: The burden of carcinoid syndrome (CS) among patients with neuroendocrine tumors is substantial and has been shown to result in increased healthcare resource use and costs. The incremental burden of CS diarrhea (CSD) is less well understood, particularly among working age adults who make up a large proportion of the population of patients with CS.

Aim: To estimate the direct medical costs of CSD to a self-insured employer in the United States. Read More

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December 2019

Carcinoid Heart Disease: a Comprehensive Review.

Curr Cardiol Rep 2019 11 19;21(11):140. Epub 2019 Nov 19.

Department of Cardiology, Division of Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Purpose Of Review: Carcinoid heart disease is a rare disorder that is associated with significant morbidity and mortality. In this review of the literature, we will present current concepts in diagnosis and management of carcinoid heart disease.

Recent Findings: Recent expert consensus guidelines highlight the role of echocardiography and screening with NT-proBNP for the evaluation of carcinoid heart disease. Read More

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November 2019

Pheochromocytoma: Another Neuroendocrine Tumor that Substantially Affects the Heart.

Run Yu

Tex Heart Inst J 2019 06 1;46(3):233. Epub 2019 Jun 1.

Division of Endocrinology, UCLA David Geffen School of Medicine, Los Angeles, California.

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Management of Gastrointestinal Neuroendocrine Tumors.

Clin Med Insights Endocrinol Diabetes 2019 24;12:1179551419884058. Epub 2019 Oct 24.

Department of Surgery, The University of Alabama at Birmingham, Birmingham, AL, USA.

Neuroendocrine neoplasms (NENs) are derived from neuroendocrine cell system and can have benign or malignant characteristics. They are rare tumors, but have been increasing in incidence over the past 40 years. Patients with NENs may develop symptoms due to primary tumor invasion, metastasis, or from secretion of hormonally active tumor substances. Read More

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October 2019

Analysis of Real-World Treatment Patterns, Healthcare Resource Utilization, and Costs Between Octreotide and Lanreotide Among Patients With Neuroendocrine Tumors.

Pancreas 2019 10;48(9):1126-1135

Division of Gastroenterology, Department of Medicine, Mount Sinai School of Medicine, New York, NY.

Objective: The aim of the study was to assess treatment patterns, healthcare resource utilization, and healthcare costs among patients with neuroendocrine tumors (NETs) receiving long-acting octreotide versus lanreotide, overall and in patients with carcinoid syndrome (CS).

Methods: A provider-based claims database was used to identify NET patients who first initiated long-acting octreotide or lanreotide (index date) from January 2015 to November 2017. Propensity-score matching 1:1 was used. Read More

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October 2019