2,383 results match your criteria Malignant Carcinoid Syndrome


Carcinoid Heart Disease: Review of Current Knowledge.

Tex Heart Inst J 2019 02 1;46(1):21-27. Epub 2019 Feb 1.

Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial pathophysiology, and the right side of the heart is usually involved. Symptoms and signs vary depending upon the affected cardiac components; most typical is right-sided heart failure secondary to diseased tricuspid and pulmonary valves. Read More

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http://dx.doi.org/10.14503/THIJ-17-6562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378997PMC
February 2019
1 Read

Long-Term Safety Experience with Telotristat Ethyl Across Five Clinical Studies in Patients with Carcinoid Syndrome.

Oncologist 2019 Jan 16. Epub 2019 Jan 16.

Lexicon Pharmaceuticals, Inc., The Woodlands, Texas, USA

Background: Patients with neuroendocrine tumors (NETs) and carcinoid syndrome experience considerable morbidity and mortality; carcinoid syndrome may be associated with shorter survival. Carcinoid syndrome is linked to tumoral secretion of serotonin and other bioactive substances. The subsequent debilitating diarrhea and urgency to defecate pose significant health risks. Read More

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http://dx.doi.org/10.1634/theoncologist.2018-0236DOI Listing
January 2019
3 Reads

Carcinoid heart disease involving the left heart: a case report and biomarker analysis.

ESC Heart Fail 2019 02 8;6(1):222-227. Epub 2019 Jan 8.

Department of Internal Medicine, Oberndorf Hospital, Salzburg, Austria.

Herein, we report the case of a 67-year-old woman who was admitted to our hospital because of dyspnoea and oedema of the lower extremities. Transthoracic echocardiography revealed severe tricuspid and mitral regurgitation, and the leaflets of the tricuspid valve were found to be rigid and almost immobile. The plasma concentrations of serotonin and chromogranin A were elevated, and hence, suspicion for carcinoid heart disease was raised. Read More

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http://doi.wiley.com/10.1002/ehf2.12396
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http://dx.doi.org/10.1002/ehf2.12396DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352891PMC
February 2019
14 Reads

Multiple neuroendocrine tumor of the small bowel: a case report and a review of literature.

Vnitr Lek 2018 ;64(10):966-969

Primary malignant tumors of small bowel constitute only about 1-2% of all gastrointestinal neoplasms. Although neuroendocrine tumors (NETs) are relatively rare, they still represent the second most common malignancy of the small bowel (after adenocarcinoma). Clinical manifestations include abdominal pain, bowel obstruction, diarrhea, weight loss and bleeding. Read More

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January 2018
11 Reads

Impact of carcinoid syndrome symptoms and long-term use of somatostatin analogs on quality of life in patients with carcinoid syndrome: A survey study.

Medicine (Baltimore) 2018 Nov;97(47):e13390

Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL.

To evaluate association of carcinoid syndrome (CS) symptom burden and somatostatin analog (SSA) duration with quality of life (QoL) using Functional Assessment of Cancer Therapy-General (FACT-G) and Patient-Reported Outcomes Measurement Information System (PROMIS-29) instruments.Adults who received treatment for CS symptoms in the US were recruited to participate in a cross-sectional online survey (July-October, 2016). Demographic, clinical, and QoL questions (FACT-G, 29 CS-related supplemental questions, PROMIS-29) were included. Read More

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http://dx.doi.org/10.1097/MD.0000000000013390DOI Listing
November 2018
10 Reads

Histopathology of Neuroendocrine Neoplasms of the Gastrointestinal System.

Klin Onkol 2018 ;31(3):167-177

Background: Tumors arising from neuroendocrine cells are defined as epithelial neoplasms with predominantly neuroendocrine differentiation. They comprise a distinct group of tumors with a characteristic histological structure and functional properties that develop at various sites, particularly the gastrointestinal system (67%) and lungs (25%). Although such tumors are usually slow-growing and indolent, almost all have malignant potential and most can produce active hormones. Read More

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http://dx.doi.org/10.14735/amko2018167DOI Listing
January 2018
1 Read

Cutaneous Metastasis of Gastroenteropancreatic Neuroendocrine Tumors (GEP-Nets).

JOP 2018 Sep 18;19(5). Epub 2018 Sep 18.

The Department of Medicine, Tufts Medical Center, Boston MA, USA.

Background: Gastroenteropancreatic neuroendocrine tumors are neoplasms commonly found within the gastrointestinal tract that originate from endocrine cells. These are slow progressive tumors and often metastasize to other elements of the gastrointestinal tract including the liver. Consequently, these tumors release hormones including serotonin and/or histamine that are responsible for the symptoms including intermittent flushing and diarrhea. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6217987PMC
September 2018
12 Reads

Expanding the phenotype of COPA syndrome: a kindred with typical and atypical features.

J Med Genet 2018 Nov 1. Epub 2018 Nov 1.

Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.

Background: Copa syndrome is a rare autosomal dominant disorder with abnormal intracellular vesicle trafficking. The objective of this work is to expand the knowledge about this disorder by delineating phenotypic features of an unreported COPA family.

Methods And Results: A heterozygous missense variant (c. Read More

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http://jmg.bmj.com/lookup/doi/10.1136/jmedgenet-2018-105560
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http://dx.doi.org/10.1136/jmedgenet-2018-105560DOI Listing
November 2018
16 Reads

An update of lanreotide acetate for treatment of adults with carcinoid syndrome.

Drugs Today (Barc) 2018 Aug;54(8):457-465

Yale Cancer Center, Smilow Cancer Hospital, New Haven, Connecticut, USA.

Carcinoid tumors are rare and usually slow-growing. Some patients with advanced metastatic disease however can develop symptoms of carcinoid syndrome, which results in debilitating diarrhea and flushing. Many treatments including chemotherapy were tried unsuccessfully in the past to treat this syndrome. Read More

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http://dx.doi.org/10.1358/dot.2018.54.8.2834461DOI Listing

Quadruple Valve Replacement for Carcinoid Heart Disease.

Braz J Cardiovasc Surg 2018 Jul-Aug;33(4):398-403

Freeman Hospital Newcastle, United Kingdom of Great Britain and Northern Ireland.

Introduction: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. Read More

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http://dx.doi.org/10.21470/1678-9741-2017-0224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6122765PMC
January 2019
3 Reads

Carcinoid syndrome: update on the pathophysiology and treatment.

Clinics (Sao Paulo) 2018 08 20;73(suppl 1):e490s. Epub 2018 Aug 20.

Disciplina de Radiologia e Oncologia, Instituto do Cancer do Estado de Sao Paulo (ICESP), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.

Approximately 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 syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, 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. Read More

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http://dx.doi.org/10.6061/clinics/2018/e490sDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6096975PMC
August 2018
13 Reads

Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus.

Respir Med Case Rep 2018 10;25:129-132. Epub 2018 Aug 10.

Division of Pulmonary Medicine, Mayo Clinic Hospital, Phoenix, AZ, USA.

Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS, the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.08.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6098213PMC
August 2018
7 Reads

Carcinoid Heart Disease: A Review.

Endocrinol Metab Clin North Am 2018 09;47(3):671-682

Neuroendocrine Tumour Unit, Royal Free Hospital, Pond Street, London NW3 2QG, UK. Electronic address:

Carcinoid heart disease remains a major cause of morbidity and mortality among patients with carcinoid syndrome and metastatic neuroendocrine tumors. Screening of all patients with N-terminal pro-B-type natriuretic peptide and transthoracic echocardiography is critical for early detection, as early symptoms and signs have low sensitivity for the disease. Cardiac surgery, in appropriate cases, is the only definitive therapy for advanced carcinoid heart disease, and it improves patient symptoms and survival. Read More

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http://dx.doi.org/10.1016/j.ecl.2018.04.012DOI Listing
September 2018
5 Reads

New Treatments for the Carcinoid Syndrome.

Endocrinol Metab Clin North Am 2018 09 11;47(3):557-576. Epub 2018 Jul 11.

Department of Medicine, David Geffen School of Medicine, University of California, 700 Tiverton Avenue, Los Angeles, CA 90095, USA; Department of Neurosurgery, David Geffen School of Medicine, University of California, 700 Tiverton Avenue, Los Angeles, CA 90095, USA. Electronic address:

Neuroendocrine tumors, including carcinoids, are rare and insidiously growing tumors. Related to their site of origin, tumors can be functional, causing various forms of the carcinoid syndrome, owing to the overproduction of serotonin, histamine, or other bioactive substances. They often invade adjacent structures or metastasize to the liver and elsewhere. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08898529183052
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http://dx.doi.org/10.1016/j.ecl.2018.04.014DOI Listing
September 2018
21 Reads

Partial hepatic resections for metastatic neuroendocrine tumors: perioperative outcomes.

J Clin Anesth 2018 Dec 8;51:93-96. Epub 2018 Aug 8.

Mayo Clinic, Rochester, MN, USA.

Study Objective: Partial hepatic resection reduces tumor burden in patients with metastatic neuroendocrine tumors, thereby improving quality and length of life. These procedures can be challenging as well as life-threatening. Our aim was to evaluate our patients' perioperative outcomes and propose a definition for an intraoperative carcinoid crisis relevant to this surgery, given its unique surgical considerations. Read More

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http://dx.doi.org/10.1016/j.jclinane.2018.08.005DOI Listing
December 2018
15 Reads

Economic analysis of inadequate symptom control in carcinoid syndrome in the United States.

Future Oncol 2018 Oct 10;14(23):2361-2370. Epub 2018 Aug 10.

Lexicon Pharmaceuticals, Inc., The Woodlands, TX 77381, USA.

Aim: We investigated the healthcare resource utilization and costs of patients with dose escalations beyond recommended levels of long-acting octreotide for persistent carcinoid syndrome (CS) symptoms.

Materials & Methods: A retrospective study of US health insurance claims included 358 adults with ≥1 medical claim for CS and ≥6 claims for long-acting octreotide (10-30 mg) between 1 July 2006 and 31 December 2013.

Results: Pre-escalation per-patient per-month outcomes and costs were lower versus post-escalation, including vascular conditions (0. Read More

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https://www.futuremedicine.com/doi/10.2217/fon-2018-0129
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http://dx.doi.org/10.2217/fon-2018-0129DOI Listing
October 2018
8 Reads

Telotristat ethyl for the treatment of carcinoid syndrome diarrhea not controlled by somatostatin analogues.

Authors:
P M Kasi

Drugs Today (Barc) 2018 Jul;54(7):423-432

College of Medicine and Oncology, Division of Hematology/Oncology, Mayo Clinic, Jacksonville, Florida, USA.

Telotristat ethyl (Xermelo), developed by Lexicon Pharmaceuticals, is an oral tryptophan hydroxylase inhibitor blocking peripheral conversion of tryptophan to serotonin (5-hydroxytryptamine [5-HT]). It was approved by the U.S. Read More

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http://dx.doi.org/10.1358/dot.2018.54.7.2834460DOI Listing
July 2018
1 Read

Exercise cardiac magnetic resonance imaging with pulmonary artery catheter monitoring in carcinoid heart disease: a shift towards early intervention?

ESC Heart Fail 2018 10 6;5(5):953-955. Epub 2018 Aug 6.

Division of Cardiology, University Hospital Leuven, Herestraat 49, 3000, Leuven, Belgium.

Neuroendocrine tumours are a rare malignancy, which can be complicated by a carcinoid syndrome and, in more rare cases, also valve destruction. The correct timing for surgical repair remains unknown. We report the first-in-men exercise cardiac magnetic resonance imaging with pulmonary artery catheter measurements in order to better understand the haemodynamic impact of isolated tricuspid valve insufficiency in a low symptomatic patient. Read More

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http://dx.doi.org/10.1002/ehf2.12328DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6165946PMC
October 2018
2 Reads

Grade 3 Neuroendocrine Tumor (G3 NET) in a Background of Multiple Serotonin Cell Neoplasms of the Ileum Associated with Carcinoid Syndrome and Aggressive Behavior.

Endocr Pathol 2018 Dec;29(4):369-373

Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100, Pavia, Italia.

Grade 3 well-differentiated neuroendocrine tumors (G3 NETs) have been characterized in the pancreas and stomach and distinguished from low-to-intermediate grade (G1-G2) NETs, as well as from highly malignant, poorly differentiated neuroendocrine carcinomas (NECs). Up to now, no G3 NET has been thoroughly described in the distal small intestine. We herein report a case of a 61-year-old man presenting with carcinoid syndrome. Read More

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http://dx.doi.org/10.1007/s12022-018-9541-8DOI Listing
December 2018
7 Reads

Endocrine paraneoplastic syndromes in lung cancer.

Hormones (Athens) 2018 Sep 2;17(3):351-358. Epub 2018 Jul 2.

Pulmonary Department, G. Papanikolaou Hospital, Aristotle University of Thessaloniki, Exohi, 570 10, Thessaloniki, Greece.

Paraneoplastic syndromes are defined as a combination of clinical disorders associated with malignant diseases that are caused by the secretion of various substances by the tumor without, however, being caused by the direct growth and infiltration of the primary tumor, or due to the development of distant metastases. Despite the fact that lung cancer represents the number one cause of death from cancer worldwide, the new methods of treatment increase patient survival and the incidence of paraneoplastic syndromes. The most important ones of these are humoral hypercalcemia of malignancy, syndrome of inappropriate antidiuretic hormone, hyponatremia of malignancy, ectopic Cushing's syndrome, carcinoid syndrome, and hypoglycemia and are usually a poor prognostic marker. Read More

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http://dx.doi.org/10.1007/s42000-018-0046-0DOI Listing
September 2018
13 Reads

Right and left-sided carcinoid heart disease.

Cardiol J 2018 ;25(3):417

Department of Radiology, Institute of Cardiology,, Alpejska 42, 04-628 WARSAW, Poland.

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http://dx.doi.org/10.5603/CJ.2018.0061DOI Listing
December 2018
2 Reads

Somatostatin Receptor PET/CT Features of Carcinoid Heart Disease.

Clin Nucl Med 2018 Aug;43(8):e280-e281

From the Departments of Nuclear Medicine.

We present the case of a 35-year-old woman with metastatic neuroendocrine tumor undergoing treatment with long-acting octreotide and Lu-DOTATATE therapy. We present features of carcinoid heart disease on Ga-DOTANOC PET/CT, which revealed dilated right atrium, pericardial effusion, ascites, and congestive hepatopathy apart from the metastatic lesions in the liver. The scan cardiac findings were confirmed by echocardiography. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002139DOI Listing
August 2018
29 Reads
3.931 Impact Factor

Peptide Receptor Radionuclide Therapy with Lu-DOTATATE in Carcinoid Heart Disease: A Contraindication or a Promising Treatment Approach Bettering Chances for Corrective Surgery?

J Nucl Med Technol 2018 Sep 8;46(3):292-294. Epub 2018 Jun 8.

Radiation Medicine Centre (BARC), Tata Memorial Hospital Annexe, Parel, India; and Homi Bhabha National Institute, Mumbai, India

We report the gratifying response of functioning metastatic neuroendocrine tumor with carcinoid heart disease (uncontrolled by long-acting octreotide) to treatment with Lu-DOTATATE. This response favorably altered the clinical course of the patient, enabling corrective valvular surgery, enhancing health-related quality of life, improving symptoms (from New York Heart Association grade III at baseline to grade I after 6 cycles), stabilizing the disease, and substantially reducing the level of 5-hydroxyindole acetic acid. Avoiding concerns about volume overload by administering amino acids over a relatively prolonged period of 7. Read More

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http://tech.snmjournals.org/lookup/doi/10.2967/jnmt.118.2101
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http://dx.doi.org/10.2967/jnmt.118.210179DOI Listing
September 2018
25 Reads

[Heart failure manifestation of carcinoid tricuspid dysfunction].

Kardiol Pol 2018 ;76(4):808

I Cardiology Clinic, District Hospital, Kielce.

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http://dx.doi.org/10.5603/KP.2018.0082DOI Listing
December 2018
3 Reads

Management of functional neuroendocrine tumors of the pancreas.

Authors:
Kjell Öberg

Gland Surg 2018 Feb;7(1):20-27

Department of Medical Sciences, Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden.

Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Read More

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http://dx.doi.org/10.21037/gs.2017.10.08DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5876681PMC
February 2018
20 Reads

Antidepressants appear safe in patients with carcinoid tumor: Results of a retrospective review.

Eur J Surg Oncol 2018 06 21;44(6):744-749. Epub 2018 Mar 21.

Department of Psychiatry and Behavioral Sciences, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, 10065, NY, USA. Electronic address:

Introduction: Patients living with neuroendocrine tumors have high rates of depression, often necessitating antidepressants, including selective serotonin reuptake inhibitors (SSRI). Neuroendocrine tumors (NETs) secrete vasoactive substances, including serotonin, which contribute to the cluster of symptoms known as carcinoid syndrome (flushing and diarrhea). Controversy exists over whether or not antidepressants are safe in NET. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07487983183095
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http://dx.doi.org/10.1016/j.ejso.2018.03.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5970966PMC
June 2018
17 Reads

LANREOTIDE THERAPY IN CARCINOID SYNDROME: PROSPECTIVE ANALYSIS OF PATIENT-REPORTED SYMPTOMS IN PATIENTS RESPONSIVE TO PRIOR OCTREOTIDE THERAPY AND PATIENTS NAÏVE TO SOMATOSTATIN ANALOGUE THERAPY IN THE ELECT PHASE 3 STUDY.

Endocr Pract 2018 Mar;24(3):243-255

Objective: This ELECT prospective analysis examined lanreotide depot/autogel for carcinoid syndrome (CS) symptom control in patients with neuroendocrine tumors (NETs) who were responsive to prior octreotide (prior octreotide group) compared with patients who were naïve to prior somatostatin analogue treatment (de novo group).

Methods: Adults with histopathologically confirmed NET and stable CS (diarrhea and/or flushing) were randomized to subcutaneous (SC) lanreotide 120 mg or placebo every 4 weeks for 16 weeks. Patients reported diarrhea and/or flushing symptom severity and frequency and short-acting SC octreotide rescue therapy daily using an Interactive Voice/Web Response System. Read More

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http://dx.doi.org/10.4158/EP172000.ORDOI Listing
March 2018
16 Reads

A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves.

Am J Case Rep 2018 Mar 12;19:284-288. Epub 2018 Mar 12.

Department of Medicine, Krannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, IN, USA.

BACKGROUND Carcinoid tumor, benign, low-grade malignant, and high-grade malignant, can be associated with the release of vasoactive substances that cause symptoms including cutaneous flushing, diarrhea, and bronchospasm. In 50-60% of patients with carcinoid syndrome, the vasoactive substances cause fibrosis of the pulmonary and tricuspid heart valves which lead to regurgitation and right-sided heart failure. The right side of the heart is usually affected because monoamine oxidases in the lungs usually inactivate the vasoactive substances. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5865407PMC
March 2018
6 Reads

Carcinoid Heart Disease: Early Outcomes after Surgical Valve Replacement in Nine Patients.

Heart Surg Forum 2018 02 16;21(1):E040-E043. Epub 2018 Feb 16.

Department of Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, United Kingdom.

Aim: To describe the early outcomes of carcinoid patients undergoing surgical heart valve replacement.

Methods: In a retrospective study, records of patients with symptomatic carcinoid heart disease referred for valve surgery between 2012 and 2016 were reviewed. The perioperative and early postoperative outcomes were analyzed. Read More

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http://dx.doi.org/10.1532/hsf.1855DOI Listing
February 2018
4 Reads

Health-related quality of life, anxiety, depression and impulsivity in patients with advanced gastroenteropancreatic neuroendocrine tumours.

World J Gastroenterol 2018 Feb;24(6):671-679

Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester M20 4BX, United Kingdom.

Aim: To compare health-related quality of life (HRQoL), anxiety, depression, and impulsivity scores in patients with and without carcinoid syndrome (CS), and correlated them with serum 5-hydroxyindoleacetic acid (5-HIAA) levels.

Methods: Patients with advanced gastroenteropancreatic neuroendocrine tumours (GEPNET), with and without CS completed HRQoL QLQ-C30 and QLQ-GI.NET21, Hospital Anxiety and Depression Scale (HADS) and Barratt Impulsivity Scale (BIS) questionnaires. Read More

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http://www.wjgnet.com/1007-9327/full/v24/i6/671.htm
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http://dx.doi.org/10.3748/wjg.v24.i6.671DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5807670PMC
February 2018
24 Reads

The Role of 5-HT1A Receptor in Cancer as a New Opportunity in Medicinal Chemistry.

Curr Med Chem 2018 ;25(27):3214-3227

Dipartimento di Farmacia, Universita degli Studi di Napoli Federico II - Via D. Montesano, 49 - 80131, Napoli, Italy.

The 5-HT1A receptor is a pharmacologically well characterized serotonin receptor subtype and it has long been investigated because of its involvement in several physiopathological mechanisms and treatment of neurological diseases like ansia and depression. Serotonin (5-HT) also shows many non-neural functions such as essential hypertension, embryogenesis, follicle maturation and behavior. Moreover, it exerts a growth factor function on different types of non-tumoral cells, and it was also found to be related to oncogenes. Read More

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http://dx.doi.org/10.2174/0929867325666180209141650DOI Listing
September 2018
8 Reads

The Perfect Storm: Carcinoid Heart Disease and Acute Right Ventricular Failure.

J Cardiothorac Vasc Anesth 2018 04 4;32(2):846-847. Epub 2018 Jan 4.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1053/j.jvca.2017.12.053DOI Listing
April 2018
4 Reads

4-Valve Heart Disease and Right Heart Failure.

J Cardiothorac Vasc Anesth 2018 04 13;32(2):838-845. Epub 2017 Oct 13.

Emory University Hospital, Emory University School of Medicine, Atlanta, GA.

Carcinoid heart disease is a rare form of heart disease due to secretion of vasoactive compounds, including serotonin, from gastrointestinal tumors. This E-challenge examines the case of a patient with advanced carcinoid heart disease who presented to the operating room (OR) for a tricuspid valve replacement. Once the patient was in the OR, intraoperative transesophageal echocardiography was used to discover a patent foramen ovale and involvement of all 4 valves with regurgitant lesions. Read More

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http://dx.doi.org/10.1053/j.jvca.2017.10.019DOI Listing
April 2018
26 Reads

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report.

J Med Case Rep 2018 Jan 31;12(1):23. Epub 2018 Jan 31.

Department of Cardiology, Ibn Rushd University Hospital, Casablanca, Morocco.

Background: Carcinoid heart disease is a frequent complication of carcinoid syndrome. It is related to the release by the carcinoid tumor and/or its metastases of bioactive substances such as serotonin. It is characterized by right-sided valvular involvement and can lead to right-sided heart failure. Read More

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http://dx.doi.org/10.1186/s13256-018-1574-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791312PMC
January 2018
10 Reads

Telotristat ethyl: a novel agent for the therapy of carcinoid syndrome diarrhea.

Future Oncol 2018 May 19;14(12):1155-1164. Epub 2018 Jan 19.

Division of Hematology Oncology, University of Iowa Hospital & VA Medical Center, Iowa City, IA 52242, USA.

Carcinoid syndrome (CS), characterized by diarrhea and flushing, is present in 20% of patients with neuroendocrine tumors at diagnosis and becomes more frequent with progression. The diarrhea of CS is caused mainly by tumoral secretion of serotonin. It may not be fully controlled by somatostatin analogs, the currently indicated drugs for symptomatic relief. Read More

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http://dx.doi.org/10.2217/fon-2017-0340DOI Listing
May 2018
4 Reads

Telotristat ethyl in carcinoid syndrome: safety and efficacy in the TELECAST phase 3 trial.

Endocr Relat Cancer 2018 03 12;25(3):309-322. Epub 2018 Jan 12.

Oncology DepartmentHospital Universitario 12 de Octubre, Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), UCM, CNIO, CIBERONC, Madrid, Spain.

Telotristat ethyl, a tryptophan hydroxylase inhibitor, was efficacious and well tolerated in the phase 3 TELESTAR study in patients with carcinoid syndrome (CS) experiencing ≥4 bowel movements per day (BMs/day) while on somatostatin analogs (SSAs). TELECAST, a phase 3 companion study, assessed the safety and efficacy of telotristat ethyl in patients with CS (diarrhea, flushing, abdominal pain, nausea or elevated urinary 5-hydroxyindoleacetic acid (u5-HIAA)) with <4 BMs/day on SSAs (or ≥1 symptom or ≥4 BMs/day if not on SSAs) during a 12-week double-blind treatment period followed by a 36-week open-label extension (OLE). The primary safety and efficacy endpoints were incidence of treatment-emergent adverse events (TEAEs) and percent change from baseline in 24-h u5-HIAA at week 12. Read More

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http://dx.doi.org/10.1530/ERC-17-0455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5811631PMC
March 2018
11 Reads

Anesthetic Management of Patients With Carcinoid Syndrome and Carcinoid Heart Disease: The Mount Sinai Algorithm.

J Cardiothorac Vasc Anesth 2018 04 20;32(2):1023-1031. Epub 2017 Nov 20.

Department of Anesthesiology, Division of Cardiac Anesthesia, The Mount Sinai Hospital, Mount Sinai Health System, New York, NY.

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http://dx.doi.org/10.1053/j.jvca.2017.11.027DOI Listing
April 2018
7 Reads

Telotristat Ethyl for Patients With Carcinoid Syndrome Associated With Chest Pain and Hypertension.

Pancreas 2018 01;47(1):e2

Division of Hematology/Oncology Mayo Clinic, FL

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http://dx.doi.org/10.1097/MPA.0000000000000971DOI Listing
January 2018
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Budgetary Impact of Telotristat Ethyl, a Novel Treatment for Patients with Carcinoid Syndrome Diarrhea: A US Health Plan Perspective.

Clin Ther 2017 Dec 23;39(12):2338-2344. Epub 2017 Nov 23.

Lexicon Pharmaceuticals, Inc, Basking Ridge, New Jersey.

Purpose: Telotristat ethyl (TE) was recently approved for carcinoid syndrome diarrhea (CSD) in patients not adequately controlled with somatostatin analog long-acting release (SSA LAR) therapy alone. A budget impact model was developed to determine the short-term affordability of reimbursing TE in a US health plan.

Methods: A budget impact model compared health care costs when CSD is managed per current treatment patterns (SSA LAR, reference drug scenario) versus when TE is incorporated in the treatment algorithm (SSA LAR + TE, new drug scenario). Read More

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http://dx.doi.org/10.1016/j.clinthera.2017.10.019DOI Listing
December 2017
5 Reads

Management of Well-differentiated Gastroenteropancreatic Neuroendocrine Tumors (GEPNETs): A Review.

Clin Ther 2017 Nov;39(11):2146-2157

Allegheny Health Network Cancer Institute, Allegheny General Hospital, Pittsburgh, Pennsylvania. Electronic address:

Purpose: Neuroendocrine tumors (NETs) are heterogeneous tumors that arise from the neuroendocrine cells of the digestive tract and other organs, such as the lung, ovary, and thyroid glands. They can be well differentiated or poorly differentiated, and management of these tumors differs for each histologic subtype. We have performed a review of NETs and focused on management of well-differentiated gastroenteropancreatic neuroendocrine tumors (GEPNETs) and carcinoid syndrome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01492918173101
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http://dx.doi.org/10.1016/j.clinthera.2017.10.010DOI Listing
November 2017
8 Reads

Predictive factors associated with carcinoid syndrome in patients with gastrointestinal neuroendocrine tumors.

World J Gastroenterol 2017 Oct;23(40):7283-7291

Division of Gastroenterology, University of Pennsylvania Health System, 3400 Civic Center Boulevard, Perelman Center for Advanced Medicine, Philadelphia, PA 19104, United States.

Aim: To discover unknown factors associated with carcinoid syndrome (CS) with the goal of earlier diagnosis of CS.

Methods: In this retrospective case-control study using United States administrative claims, patients (≥ 18 years) newly-diagnosed with gastrointestinal neuroendocrine tumors (GI NETs) without CS (controls) were exactly matched to patients with CS (cases) based on NET diagnosis date at a 3-to-1 ratio. Study index date was first CS diagnosis (controls: same distance from NET diagnosis as cases). Read More

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http://dx.doi.org/10.3748/wjg.v23.i40.7283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5677203PMC
October 2017
11 Reads

Carcinoid-syndrome: recent advances, current status and controversies.

Curr Opin Endocrinol Diabetes Obes 2018 02;25(1):22-35

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

Purpose Of Review: To review recent advances and controversies in all aspects of carcinoid-syndrome.

Recent Findings: Over the last few years there have been a number of advances in all aspects of carcinoid syndrome as well as new therapies. These include new studies on its epidemiology which demonstrate it is increasing in frequency; increasing insights into the pathogenesis of its various clinical manifestations and into its natural history: definition of prognostic factors; new methods to verify its presence; the development of new drugs to treat its various manifestations, both initially and in somatostatin-refractory cases; and an increased understanding of the pathogenesis, natural history and management of carcinoid heart disease. Read More

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http://dx.doi.org/10.1097/MED.0000000000000376DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5747542PMC
February 2018
7 Reads

Carcinoid Valve Disease: A Case Report and Review.

J Heart Valve Dis 2017 05;26(3):321-326

Division of Cardiology, UCLA Medical Center, Los Angeles, CA, USA.

In patients with carcinoid syndrome, the development of carcinoid valve disease typically carries an unfavorable prognosis. We present the case of a patient with significant valvular dysfunction secondary to carcinoid valve disease. Valve replacement surgery was complicated by the development of prosthetic valve degeneration, ultimately requiring percutaneous valve implantation in a valve-in-valve fashion. Read More

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May 2017
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Echocardiography in functional midgut neuroendocrine tumors: When and how often.

Rev Endocr Metab Disord 2017 Dec;18(4):411-421

Department of Cardiovascular Surgery, Mount Sinai Health System, The Mount Sinai Hospital, 1190 Fifth Avenue, GP2 West, New York, NY, 10029-6574, USA.

The management of patients with midgut neuroendocrine tumors (MNET) is rapidly evolving. Current preoperative detection rates of primary tumor sites are higher than ever and progression-free survival in patients with already advanced disease is expanding due to the implementation of novel efficacious treatment strategies. This survival benefit may potentially translate into a need for a multidisciplinary approach to an even more heterogenous variety of clinical conditions, among these, carcinoid syndrome (CS) and carcinoid heart disease (CHD). Read More

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http://dx.doi.org/10.1007/s11154-017-9434-zDOI Listing
December 2017
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Understanding the Patient Experience with Carcinoid Syndrome: Exit Interviews from a Randomized, Placebo-controlled Study of Telotristat Ethyl.

Clin Ther 2017 Nov 23;39(11):2158-2168. Epub 2017 Oct 23.

RTI Health Solutions, Research Triangle Park, North Carolina.

Purpose: Telotristat ethyl, an oral tryptophan hydroxylase inhibitor, is intended to treat carcinoid syndrome by reducing serotonin production. Telotristat ethyl was evaluated in TELESTAR, a Phase III study for patients who had carcinoid syndrome with at least 4 bowel movements (BMs) per day and who were receiving somatostatin analogue therapy. This interview substudy was conducted to provide insight into the patient experience in TELESTAR and to help understand whether reductions in BM frequency (the primary end point) and other symptoms were clinically meaningful. Read More

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http://dx.doi.org/10.1016/j.clinthera.2017.09.013DOI Listing
November 2017
31 Reads

Transcatheter valve implantation to inferior vena cava to control carcinoid symptoms.

BMJ Case Rep 2017 Oct 23;2017. Epub 2017 Oct 23.

Department of Liver Medicine, University Hospitals Birmingham, NHS Foundation Trust, Birmingham, UK.

Severe carcinoid syndrome and carcinoid heart disease in neuroendocrine tumours can have a significant impact on a patient's quality of life and are a major cause of morbidity and mortality. We present a novel approach to managing a patient with medically uncontrollable carcinoid syndrome. Inferior and superior vena cava placement of transcatheter heart valves has been used to treat patients with right heart failure due to severe tricuspid and pulmonary regurgitation. Read More

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http://dx.doi.org/10.1136/bcr-2017-220888DOI Listing
October 2017
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Telotristat ethyl: proof of principle and the first oral agent in the management of well-differentiated metastatic neuroendocrine tumor and carcinoid syndrome diarrhea.

Cancer Chemother Pharmacol 2017 Dec 19;80(6):1055-1062. Epub 2017 Oct 19.

Section of GI Cancers and Experimental Therapeutics, Division of GI Oncology and Experimental Therapeutics, Tufts Medical Center, Tufts University School of Medicine, 800 Washington Street, Boston, MA, 02111, USA.

Introduction: Metastatic neuroendocrine tumors (NETs) are associated with carcinoid syndrome that is typically characterized by diarrhea, cutaneous flushing and bronchospasm. Treatment with somatostatin analogues (SSA) improves the symptom burden but a significant proportion of patients stop responding to SSA therapy eventually. Novel agents with the potential to effectively control the symptoms are urgently needed. Read More

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http://dx.doi.org/10.1007/s00280-017-3462-yDOI Listing
December 2017
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Carcinoid Syndrome Complicating a Pancreatic Neuroendocrine Tumor: A Case Report.

Pancreas 2017 Nov/Dec;46(10):1381-1385

From the *Department of Hematology/Oncology, Fox Chase Cancer Center, Philadelphia, PA; †Division of Cardiovascular Medicine, Department of Medicine; ‡Department of Radiation Oncology, Stanford University School of Medicine, Stanford; §Department of Cardiovascular Surgery, UCSF Medical Center, San Francisco; ∥Department of Radiology (Nuclear Medicine); and ¶Department of Medicine (Division of Oncology), Stanford University School of Medicine, Stanford, CA.

Neuroendocrine tumors (NETs) comprise a heterogeneous group of neoplasms. These tumors can produce a wide variety of hormones that can lead to syndromes of hormone excess, such as carcinoid syndrome. We present the case of a 47-year-old man who presented with right upper quadrant abdominal pain and emesis. Read More

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http://dx.doi.org/10.1097/MPA.0000000000000932DOI Listing
June 2018
4 Reads

MANAGEMENT OF ENDOCRINE DISEASE: Flushing: current concepts.

Eur J Endocrinol 2017 Nov;177(5):R219-R229

Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, and NET Centre of Excellence, Royal Free London, UK.

Objective: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions.

Design And Methods: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'. Read More

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http://dx.doi.org/10.1530/EJE-17-0295DOI Listing
November 2017
19 Reads