2,431 results match your criteria Malignant Carcinoid Syndrome


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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http://dx.doi.org/10.3332/ecancer.2020.1009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7105337PMC
February 2020

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

J Gastrointest Cancer 2020 Mar 7. Epub 2020 Mar 7.

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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http://dx.doi.org/10.1007/s12029-020-00375-2DOI Listing

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

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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http://dx.doi.org/10.3748/wjg.v26.i6.686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7029351PMC
February 2020

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

Palliat Support Care 2020 Feb 10:1-7. Epub 2020 Feb 10.

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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http://dx.doi.org/10.1017/S147895152000005XDOI Listing
February 2020

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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http://dx.doi.org/10.3748/wjg.v25.i47.6857DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6931008PMC
December 2019

Carcinoid Tricuspid Valve Disease: Applications of Three Dimensional Transesophageal Echocardiography.

Circ Cardiovasc Imaging 2019 12 26;12(12):e009555. Epub 2019 Nov 26.

Cleveland Clinic Foundation, OH.

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http://dx.doi.org/10.1161/CIRCIMAGING.119.009555DOI Listing
December 2019
5 Reads

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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http://dx.doi.org/10.1007/s11886-019-1207-8DOI Listing
November 2019

Pheochromocytoma: Another Neuroendocrine Tumor that Substantially Affects the Heart.

Authors:
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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http://dx.doi.org/10.14503/THIJ-19-7024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827478PMC

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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http://dx.doi.org/10.1177/1179551419884058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6820165PMC
October 2019
1 Read

Carcinoid Heart Disease: A Guide for Clinicians.

Cardiol Clin 2019 Nov;37(4):497-503

Division of Cardiology, Department of Medicine, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA. Electronic address:

Carcinoid heart disease is the collective term for all cardiac manifestations that develop in patients with carcinoid. The cardiac manifestations of carcinoid tumors are attributed to the paraneoplastic effects of vasoactive substances released by the malignant cells. The clinical manifestations of carcinoid heart disease include valvular destruction leading to valvular regurgitation and stenosis, right-sided heart failure, and metastatic carcinoid disease. Read More

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http://dx.doi.org/10.1016/j.ccl.2019.07.014DOI Listing
November 2019
1 Read
1.064 Impact Factor

Burden of Carcinoid Heart Disease in Patients With Carcinoid Syndrome Initiating Somatostatin Analogues.

Clin Ther 2019 09 17;41(9):1716-1723.e2. Epub 2019 Jul 17.

Icahn Medical School at the Mt. Sinai Medical Center, New York, NY, USA.

Purpose: As a result of overproduction of serotonin, patients with uncontrolled carcinoid syndrome (CS) may develop carcinoid heart disease (CaHD). However, the prevalence and health care resources to manage CaHD are not well understood. This study investigated the prevalence and economic burden of CaHD among adults with CS in the United States. Read More

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http://dx.doi.org/10.1016/j.clinthera.2019.06.013DOI Listing
September 2019
6 Reads

Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis.

Front Endocrinol (Lausanne) 2019 11;10:339. Epub 2019 Jun 11.

Section on Endocrinology and Genetics and Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, United States.

Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome inherited in an autosomal dominant manner and characterized by a predisposition to a multitude of endocrine neoplasms primarily of parathyroid, enteropancreatic, and anterior pituitary origin, as well as nonendocrine neoplasms. Other endocrine tumors in MEN1 include foregut carcinoid tumors, adrenocortical tumors, and rarely pheochromocytoma. Nonendocrine manifestations include meningiomas and ependymomas, lipomas, angiofibromas, collagenomas, and leiomyomas. Read More

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http://dx.doi.org/10.3389/fendo.2019.00339DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6584804PMC
June 2019
7 Reads

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

Oncologist 2019 11 12;24(11):1446-1452. Epub 2019 Jun 12.

Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.

Background: When carcinoid syndrome (CS) diarrhea (CSD) is inadequately controlled with long-acting somatostatin analogs (SSAs), clinical practice guidelines recommend addition of the tryptophan hydroxylase inhibitor telotristat ethyl (TE). In a 12-week multinational, randomized controlled trial, TE added to SSA reduced peripheral serotonin and the frequency of CSD. We evaluated real-world effectiveness of TE using patient-reported data from a nurse support program over 3 months. Read More

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http://dx.doi.org/10.1634/theoncologist.2018-0921DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6853091PMC
November 2019
8 Reads

Systemic Treatment Options for Carcinoid Syndrome: A Systematic Review.

Oncology 2019 24;96(6):273-289. Epub 2019 Apr 24.

Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois, USA.

Background: Carcinoid syndrome symptoms significantly reduce quality of life in patients with neuroendocrine tumors. Evidence supporting the use of somatostatin analogues in carcinoid syndrome symptom control dates back 30 years. The introduction of new treatment options for carcinoid syndrome, such as telotristat ethyl in 2017, highlights the need for a review of high-level evidence of new and established systemic treatments. Read More

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http://dx.doi.org/10.1159/000499049DOI Listing
June 2019
6 Reads

Assessment of change in quality of life, carcinoid syndrome symptoms and healthcare resource utilization in patients with carcinoid syndrome.

BMC Cancer 2019 Mar 28;19(1):274. Epub 2019 Mar 28.

Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, 633 North Saint Clair St.19th Floor, Chicago, IL, 60611, USA.

Background: There is limited information on changes over time in carcinoid syndrome (CS) symptoms and quality of life (QoL). This study assessed change in CS symptoms and QoL in patients treated with somatostatin analogs (SSAs) using the Functional Assessment of Cancer Therapy-General (FACT-G) and Patient-Reported Outcomes Measurement Information System (PROMIS)-29 instruments.

Methods: Patients ≥18 years old with CS symptoms and treated with SSA or non-SSA agents in the United States were recruited through a patient advocacy group to complete a two-part, anonymous online survey. Read More

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https://bmccancer.biomedcentral.com/articles/10.1186/s12885-
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http://dx.doi.org/10.1186/s12885-019-5459-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6437890PMC
March 2019
25 Reads

Paraneoplastic syndromes and other systemic disorders associated with neuroendocrine neoplasms.

Semin Diagn Pathol 2019 Jul 18;36(4):229-239. Epub 2019 Mar 18.

Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114-2696, United States. Electronic address:

Neuroendocrine paraneoplastic syndromes (PNS) consist of metabolic disorders that accompany benign and malignant neoplasms but remain unrelated to mass effects or invasion by the primary tumor or its metastases. The underlying pathogenesis responsible for PNS usual clinical presentation relies on aberrant production of protein hormones, proteins and other substances by the tumor. Prompt recognition of characteristic signs and symptoms combined with serological identification of key substances may result in early diagnosis of PNS and its underlying malignancy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07402570193002
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http://dx.doi.org/10.1053/j.semdp.2019.03.002DOI Listing
July 2019
38 Reads

When right heart valves are open 24/7.

Turk Kardiyol Dern Ars 2019 03;47(2):156

Department of Cardiology, King's College Hospital, London, United Kingdom.

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http://dx.doi.org/10.5543/tkda.2018.01940DOI Listing
March 2019
3 Reads

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
6 Reads

Generalized Cytokine Increase in the Setting of a Multisystem Clinical Disorder and Carcinoid Syndrome Associated with a Novel NLRP12 Variant.

Dig Dis Sci 2019 08 20;64(8):2140-2146. Epub 2019 Feb 20.

Division of Gastroenterology, Hepatology and Parenteral Nutrition, Department of Veterans Affairs, David Geffen School of Medicine at UCLA, VA Greater Los Angeles Healthcare System (691/111C), 11301 Wilshire Blvd., Los Angeles, CA, 90073, USA.

Background: Nucleotide-binding oligomerization domain (NOD)-like receptors (NLRs) are a group of cytoplasmic sensors that survey danger signals released by invading pathogens or damaged tissue. Mutations in the NLRP subfamily affect pro-inflammatory mediators and cause nonspecific systemic symptoms.

Aims: We sought to identify a potential genetic etiology of an inflammatory syndrome in a patient that presented with an atypical multisystem illness with carcinoid syndrome as well as atopic and autoimmune features. Read More

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http://dx.doi.org/10.1007/s10620-019-05525-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707534PMC
August 2019
10 Reads

Cardiac Metastases of Small-Bowel Carcinoid.

Circ Cardiovasc Imaging 2019 02;12(2):e008405

Biophysics and Nuclear Medicine (S.E.G., C.B., A.I.), University Hospitals of Strasbourg, University of Strasbourg, France.

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http://dx.doi.org/10.1161/CIRCIMAGING.118.008405DOI Listing
February 2019
1 Read

Serotonin levels and 1-year mortality in patients with neuroendocrine tumors: a systematic review and meta-analysis.

Future Oncol 2019 Apr 8;15(12):1397-1406. Epub 2019 Feb 8.

Icahn School of Medicine, Center for Carcinoid and Neuroendocrine Tumors, Mount Sinai Health System, New York, NY 10029, USA.

Elevated serotonin in patients with neuroendocrine tumors (NETs) may impact heart failure incidence but a quantitative relationship has not been established. Systematic review and meta-analysis of studies assessing 24-h urinary 5-hydroxyindoleacetic acid (u5-HIAA) and mortality in patients with NETs (2007-2017) with a primary outcome of 1-year mortality risk and 24-h u5-HIAA. We identified 1715 records of which 12 studies including 755 patients (3442 person-years with 376 deaths) were eligible for meta-analysis. Read More

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https://www.futuremedicine.com/doi/10.2217/fon-2018-0960
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http://dx.doi.org/10.2217/fon-2018-0960DOI Listing
April 2019
3 Reads

Cost-of-illness of metastatic gastroenteropancreatic neuroendocrine tumours in Sweden-A population-based register-linkage study.

Eur J Cancer Care (Engl) 2019 Mar 16;28(2):e12983. Epub 2019 Jan 16.

Department of Surgery, Sahlgrenska University Hospital, Gothenburg, Sweden.

The objective was to estimate the cost-of-illness of grades 1 and 2 metastatic gastroenteropancreatic neuroendocrine tumours (GEP-NETs) in Sweden in 2013 in a population-based study including all patients diagnosed between 2005 and 2013. Data were obtained from national registers, and patients who utilised healthcare resources due to metastatic GEP-NETs in 2013 were included. The study included 478 patients (mean age 64 [SD=11] years, 51% men). Read More

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http://dx.doi.org/10.1111/ecc.12983DOI Listing
March 2019
4 Reads
1.762 Impact Factor

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

Oncologist 2019 08 16;24(8):e662-e670. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6693702PMC
August 2019
21 Reads

Bioprosthetic valve thrombosis in carcinoid heart disease.

Ann Card Anaesth 2019 Jan-Mar;22(1):79-82

Department of Anesthesiology and Critical Care Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA.

Tricuspid regurgitation in carcinoid syndrome leads to significant morbidity and mortality that may warrant a tricuspid valve replacement. However, for patients with high serotonin levels and known hypercoagulable risks, the optimum timing for surgery and postoperative anticoagulation approaches remain unclear. High serotonin-triggered hypercoagulability makes prosthetic valves susceptible to thrombosis. Read More

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http://www.annals.in/text.asp?2019/22/1/79/250188
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http://dx.doi.org/10.4103/aca.ACA_2_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350430PMC
April 2020
27 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
37 Reads

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

Vnitr Lek Fall 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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May 2019
36 Reads

Discussion.

Authors:

J Thorac Cardiovasc Surg 2019 07 6;158(1):106-107. Epub 2018 Dec 6.

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http://dx.doi.org/10.1016/j.jtcvs.2018.09.123DOI Listing

Improving outcome of valve replacement for carcinoid heart disease.

J Thorac Cardiovasc Surg 2019 07 3;158(1):99-107.e2. Epub 2018 Oct 3.

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

Objective: Carcinoid heart disease is characterized by tricuspid valve regurgitation and varying degrees of pulmonary valve regurgitation or stenosis. Valve replacement procedures may be complicated by systemic effects of carcinoid syndrome, as well as hepatic dysfunction and right heart failure. This study was performed to identify factors that might be associated with improving early mortality rates and late outcomes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00225223183252
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http://dx.doi.org/10.1016/j.jtcvs.2018.09.025DOI Listing
July 2019
30 Reads

Relationship Between Symptoms and Health-related Quality-of-life Benefits in Patients With Carcinoid Syndrome: Post Hoc Analyses From TELESTAR.

Clin Ther 2018 12 24;40(12):2006-2020.e2. Epub 2018 Nov 24.

Dana-Farber Cancer Institute, Boston, MA, USA.

Purpose: Patients with metastatic neuroendocrine tumors and carcinoid syndrome (CS) may experience chronic, recurring symptoms despite somatostatin analogue therapy. Little is known about the relationship between bowel movement (BM) frequency, patient-reported symptoms and health-related quality of life (QoL). Data from the TELESTAR study were used in exploratory, post hoc analyses to understand the relationship between durable reductions in BM frequency, symptom relief, and health-related QoL. Read More

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http://dx.doi.org/10.1016/j.clinthera.2018.10.008DOI Listing
December 2018
17 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6392719PMC
November 2018
17 Reads

Real-world treatment patterns, resource use and costs of treating uncontrolled carcinoid syndrome and carcinoid heart disease: a retrospective Swedish study.

Scand J Gastroenterol 2018 Dec 19;53(12):1509-1518. Epub 2018 Nov 19.

e Department of Surgery , Sahlgrenska University Hospital , Gothenburg , Sweden.

Objectives: To quantify healthcare resource use (HRU) and costs in relation to carcinoid syndrome (CS) and carcinoid heart disease (CHD) in a real-world setting, and to provide perspective on treatment patterns.

Materials And Methods: Patient data and HRU were collected retrospectively from three Swedish healthcare registers. Adult patients diagnosed with metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs) grade 1 or 2 and CS who purchased somatostatin analogs (SSAs), and experienced controlled (defined by SSAs use) and uncontrolled (defined by SSAs dose escalation) CS for ≥8 months during the study period were included. Read More

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https://www.tandfonline.com/doi/full/10.1080/00365521.2018.1
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http://dx.doi.org/10.1080/00365521.2018.1531653DOI Listing
December 2018
41 Reads
2.329 Impact Factor

Histopathology of Neuroendocrine Neoplasms of the Gastrointestinal System.

Klin Onkol Spring 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
September 2019
6 Reads

A prospective study of the pathophysiology of carcinoid crisis.

Surgery 2019 01 8;165(1):158-165. Epub 2018 Nov 8.

Division of Surgical Oncology, Oregon Health & Science University, Portland. Electronic address:

Background: Sudden massive release of serotonin, histamine, kallikrein, and bradykinin is postulated to cause an intraoperative carcinoid crisis. The exact roles of each of these possible agents, however, remain unknown. Optimal treatment will require an improved understanding of the pathophysiology of the carcinoid crisis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00396060183062
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http://dx.doi.org/10.1016/j.surg.2018.04.093DOI Listing
January 2019
43 Reads

Commentary: Dance with me to the end of love: Serotonin and the carcinoid heart.

J Thorac Cardiovasc Surg 2019 07 10;158(1):108-109. Epub 2018 Oct 10.

Division of Cardiothoracic Surgery, University of Miami Miller School of Medicine and Jackson Memorial Hospital, Miami, Fla. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2018.09.065DOI Listing
July 2019
2 Reads

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
28 Reads

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

J Med Genet 2019 11 1;56(11):778-782. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6860403PMC
November 2019
52 Reads

Influence of carcinoid syndrome on the clinical characteristics and outcomes of patients with gastroenteropancreatic neuroendocrine tumors undergoing operative resection.

Surgery 2019 03 28;165(3):657-663. Epub 2018 Oct 28.

Division of Surgical Oncology, The Ohio State University Comprehensive Cancer Center, Columbus, OH. Electronic address:

Background: The incidence, clinical characteristics, and long-term outcomes of patients with gastroenteropancreatic neuroendrocrine tumors and carcinoid syndrome undergoing operative resection have not been well characterized.

Methods: Patients undergoing resection of primary or metastatic gastroenteropancreatic neuroendrocrine tumors between 2000 and 2016 were identified from an 8-institution collaborative database. Clinicopathologic and postoperative characteristics as well as overall survival and disease-free survival were compared among patients with and without carcinoid syndrome. Read More

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http://dx.doi.org/10.1016/j.surg.2018.09.008DOI Listing
March 2019
22 Reads

Cardiac CT Angiography in Carcinoid Heart Disease.

Radiology 2019 01 30;290(1):32. Epub 2018 Oct 30.

From the Department of Medicine, Division of Cardiology, University of Chicago-NorthShore University HealthSystem, 2680 Ridge Ave, Evanston, IL 60202 (A.A.A.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (C.A.R.).

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http://dx.doi.org/10.1148/radiol.2018181966DOI Listing
January 2019
3 Reads

Carcinoid Heart Disease in Patients With Bronchopulmonary Carcinoid.

J Thorac Oncol 2018 10 7;13(10):1602-1605. Epub 2018 Jul 7.

Department of Cardiovascular Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota. Electronic address:

Introduction: The prevalence of carcinoid heart disease (CaHD) in bronchopulmonary carcinoid and its relationship with left-sided valvular disease are unknown.

Methods: All patients with a pathologic diagnosis of bronchopulmonary carcinoid and echocardiography performed at our institution between 2001 and 2016 were retrospectively reviewed. Echocardiograms were reviewed for features of CaHD including valvular leaflet thickening and retraction with resulting regurgitation and/or stenosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15560864183077
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http://dx.doi.org/10.1016/j.jtho.2018.06.023DOI Listing
October 2018
34 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
August 2018
1 Read

Cases in a series of carcinoid syndrome and carcinoid heart disease.

Cardiovasc J Afr 2018 May/Jun;29(4):e1-e7

University of KwaZulu-Natal, Durban, South Africa; Mediclinic Heart Hospital, Pretoria, South Africa; London School of Economics and Political Science, London, UK.

Although carcinoid syndrome is regarded as a rare entity, carcinoid patients with evidence of cardiac involvement show a markedly reduced survival time. Patients with advanced signs of right-sided heart failure represent a subgroup at particularly high risk. Echocardiography remains the gold standard to diagnose or confirm structural cardiac involvement in patients with underlying carcinoid disease. Read More

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http://cvja.co.za/onlinejournal/vol29/vol29_issue4/#67/z
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http://dx.doi.org/10.5830/CVJA-2018-040DOI Listing
April 2019
6 Reads

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
20 Reads

Oral Ondansetron Offers Effective Antidiarrheal Activity for Carcinoid Syndrome Refractory to Somatostatin Analogs.

Oncologist 2019 02 31;24(2):255-258. Epub 2018 Aug 31.

Division of Oncology, Department of Internal Medicine I, Medical University Vienna, Vienna, Austria

Objectives: Somatostatin analogs (SSAs) are standard for symptomatic patients with neuroendocrine tumors (NETs). However, most patients experience tachyphylaxis, and limited options exist for this so-called "refractory carcinoid syndrome." Recently, 5-HT antagonist ondansetron has been associated with reduction of bowel movement in a small series. Read More

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http://dx.doi.org/10.1634/theoncologist.2018-0191DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369958PMC
February 2019
35 Reads

Image Gallery: Flush in carcinoid syndrome.

Br J Dermatol 2018 07;179(1):e3

Hospices Civils de Lyon - Allergology and Clinical Immunology Department, Centre Hospitalier Lyon-Sud, Pierre Bénite, 69495, France.

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http://dx.doi.org/10.1111/bjd.16605DOI Listing

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
31 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
29 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
27 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
39 Reads