2,503 results match your criteria Malignant Carcinoid Syndrome


An apparent primitive mass of the mesentery: A case report.

Medicine (Baltimore) 2022 Jun 17;101(24):e29464. Epub 2022 Jun 17.

General Surgery Department, IRCCS MultiMedica, Italy.

Introduction: Neuroendocrine tumours (NETs) are rare tumors. 55% of NETs originate in the gastrointestinal tract and the liver is the most common site of distant metastases. Serum chromogranin A is the most common biomarker for assessing the extent of disease and monitoring treatment; carcinoid syndrome occurs in 19% of NETs and is characterized by chronic diarrhea or flushing. Read More

View Article and Full-Text PDF

Pulsatile Inferior Vena Cava Contrast Material Reflux in Carcinoid Heart Disease.

Radiol Imaging Cancer 2022 May;4(3):e220038

From the Department of Radiology, Mayo Clinic, 200 1st St SW, Rochester, MN 55902.

View Article and Full-Text PDF

Carcinoid Heart Disease.

N Engl J Med 2022 05 21;386(21):e56. Epub 2022 May 21.

MedStar Union Memorial Hospital, Baltimore, MD

View Article and Full-Text PDF

High-Dose Somatostatin Analogs for the Treatment of Neuroendocrine Neoplasms: where are we Now?

Curr Treat Options Oncol 2022 Jul 1;23(7):1001-1013. Epub 2022 May 1.

Medical Oncology Department, Hospital Universitario Ramón y Cajal, 28034, Madrid, Spain.

Opinion Statement: Neuroendocrine tumors (NET) represent a complex and heterogeneous group of malignancies arising from the diffuse endocrine cells and other cells derived from the neural crest. Advanced disease is observed at diagnosis in more than one-third of patients. Somatostatin analogs (SSA) are the cornerstone in advanced well-differentiated NET treatment. Read More

View Article and Full-Text PDF

Gastric neuroendocrine neoplasms: a primer for radiologists.

Abdom Radiol (NY) 2022 Apr 12. Epub 2022 Apr 12.

Department of Radiology, Michigan Medicine, University of Michigan, 1500 East Medical Center Drive, B1D502, Ann Arbor, MI, 48109, USA.

Gastric neuroendocrine neoplasms are uncommon tumors with variable differentiation and malignant potential. Three main subtypes are recognized: type 1, related to autoimmune atrophic gastritis; type 2, associated with Zollinger-Ellison and MEN1 syndrome; and type 3, sporadic. Although endoscopy alone is often sufficient for diagnosis and management of small, indolent, multifocal type 1 tumors, imaging is essential for evaluation of larger, high-grade, and type 2 and 3 neoplasms. Read More

View Article and Full-Text PDF

Dealing With the Right Side: Carcinoid Heart Disease.

J Cardiothorac Vasc Anesth 2022 07 12;36(7):2228-2231. Epub 2022 Feb 12.

Department of Anesthesia and Critical Care, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.

View Article and Full-Text PDF

Sex differences in carcinoid syndrome: A gap to be closed.

Rev Endocr Metab Disord 2022 06 16;23(3):659-669. Epub 2022 Mar 16.

Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, Naples, Italy.

The incidence of neuroendocrine neoplasms and related carcinoid syndrome (CS) has markedly increased over the last decades and women seem to be more at risk than men for developing CS. Nevertheless, very few studies have investigated sex differences in clinical presentation and outcomes of CS. However, as per other tumours, sex might be relevant in influencing tumour localization, delay in diagnosis, clinical outcomes, prognosis and overall survival in CS. Read More

View Article and Full-Text PDF

Clinicopathological characteristics and prognostic factors of patients with primary gallbladder neuroendocrine carcinomas.

J Dig Dis 2022 Mar 15;23(3):166-173. Epub 2022 Mar 15.

Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.

Objectives: Primary gallbladder neuroendocrine carcinomas (GB-NEC) are malignant neoplasms that remained to be studied. In this study we aimed to summarize their clinicopathological characteristics, effective treatment and prognostic factors for patients with GB-NEC.

Methods: Patients with GB-NEC admitted to Shanghai Jiao Tong University Affiliated Sixth People's Hospital and Renji Hospital, School of Medicine, Shanghai Jiao Tong University from October 2012 to August 2020 were enrolled. Read More

View Article and Full-Text PDF

Shifting Paradigms in the Pathophysiology and Treatment of Carcinoid Crisis.

Ann Surg Oncol 2022 May 14;29(5):3072-3084. Epub 2022 Feb 14.

Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA.

Carcinoid crisis is a potentially fatal condition characterized by various symptoms, including hemodynamic instability, flushing, and diarrhea. The incidence of carcinoid crisis is unknown, in part due to inconsistency in definitions across studies. Triggers of carcinoid crisis include general anesthesia and surgical procedures, but drug-induced and spontaneous cases have also been reported. Read More

View Article and Full-Text PDF

Ablation of typical atrial flutter as therapeutic component in carcinoid heart disease: a case report.

J Med Case Rep 2022 Feb 2;16(1):41. Epub 2022 Feb 2.

Heart Center, Segeberger Kliniken GmbH (Academic Teaching Hospital of the Universities of Kiel, Lübeck and Hamburg), Am Kurpark 1, 23795, Bad Segeberg, Schleswig-Holstein, Germany.

Background: Carcinoid heart disease is the cardiac manifestation of carcinoid syndrome. There is limited research on rhythm management in patients with carcinoid heart disease. The association of typical atrial flutter and carcinoid heart disease in particular is poorly described. Read More

View Article and Full-Text PDF
February 2022

Recent Advances in the Diagnosis and Management of Carcinoid Syndrome.

Dis Mon 2022 Jul 28;68(7):101304. Epub 2021 Dec 28.

Sri Venkateshwara Medical College, Alipiri Road, Tirupati, Andhra Pradesh 517501, India.

Carcinoid syndrome, a paraneoplastic condition linked with the release of multiple humoral factors, affects around 30-40% of patients with well-differentiated neuroendocrine tumours. Carcinoid syndrome has a major and unfavourable impact on patients' quality of life; it raises costs when compared to non-functioning neuroendocrine tumours; and it causes patients' lifestyles to alter, such as food, job, physical activity, and social life. Somatostatin analogues have been the first-line therapy for individuals with neuroendocrine tumours and carcinoid disease for decades. Read More

View Article and Full-Text PDF

Diabetes Insipidus Induced by Combination of Short-acting Octreotide and Lanreotide for Recurrent Carcinoid Crisis of Neuroendocrine Tumour: A Case Report.

J ASEAN Fed Endocr Soc 2021 6;36(2):220-222. Epub 2021 Aug 6.

Department of Medicine, Hospital Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.

Somatostatin analogue is useful in carcinoid crisis for symptom control. Optimal dosing of somatostatin analogues for carcinoid symptoms is not known. This case highlighted management issues using combination short-acting octreotide infusion with long-acting lanreotide during carcinoid crisis. Read More

View Article and Full-Text PDF

Foreword: Recent advances in diagnosis and management of carcinoid syndrome.

Authors:
Jerrold B Leikin

Dis Mon 2022 07 25;68(7):101303. Epub 2021 Dec 25.

123 West Madison Street, Suite 800, Chicago, Ill 60602, United States. Electronic address:

View Article and Full-Text PDF

Dealing With the Right Side: Carcinoid Heart Disease.

J Cardiothorac Vasc Anesth 2022 Aug 29;36(8 Pt A):2793-2802. Epub 2021 Oct 29.

Department of Anesthesiology, The Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY.

View Article and Full-Text PDF

Synoptic reporting of echocardiography in carcinoid heart disease (ENETS Carcinoid Heart Disease Task Force).

J Neuroendocrinol 2022 03 26;34(3):e13060. Epub 2021 Nov 26.

Royal Free Hospital & University College London, London, UK.

Background: This European Neuroendocrine Tumor Society (ENETS) Expert Consensus document aims to provide practical guidance and standardization for echocardiography in the screening and follow-up of carcinoid heart disease (CHD) in patients with a neuroendocrine tumour (NET) and carcinoid syndrome.

Methods: NET experts within the ENETS Carcinoid Heart Disease Task Force reviewed both general reporting guidelines and specialized scoring systems for transthoracic echocardiography (TTE) in CHD. Based on this review, a dedicated template report was designed by the multidisciplinary working group of cardiologists, oncologists, endocrinologists, gastroenterologists, surgeons and radiologists. Read More

View Article and Full-Text PDF

Progression of Carcinoid Heart Disease in the Modern Management Era.

J Am Heart Assoc 2021 12 24;10(23):e020475. Epub 2021 Nov 24.

Department of Cardiology Ambroise Paré HospitalAssistance Publique-Hôpitaux de Paris (AP-HP)Centre de référence des cardiomyopathies et des troubles du rythme cardiaque héréditaires ou raresUniversité de Versailles-Saint Quentin (UVSQ) Boulogne France.

Background The development of carcinoid heart disease (CaHD) is still relatively unclear. It is difficult to define an optimal follow-up for patients without any cardiac involvement at baseline. The aim of this study was to assess the prevalence and natural history of CaHD by annual echocardiographic examinations. Read More

View Article and Full-Text PDF
December 2021

Weight Maintenance up to 48 Weeks in Patients With Carcinoid Syndrome Treated With Telotristat Ethyl: Pooled Data From the Open-Label Extensions of the Phase III Clinical Trials TELESTAR and TELECAST.

Clin Ther 2021 10 28;43(10):1779-1785. Epub 2021 Sep 28.

The ARDEN NET Centre, ENETS Centre of Excellence, University Hospitals Coventry and Warwickshire, National Health Service Trust, Coventry, United Kingdom.

Reported incidences of neuroendocrine tumors (NETs) appear to be increasing, possibly due to greater disease awareness and increased accuracy of diagnosis. Approximately 20% of patients with NETs develop carcinoid syndrome (CS), which arises from elevated secretion of bioactive compounds, including serotonin, from NETs. This leads to symptoms including diarrhea and flushing, which result in weight loss and are associated with considerable negative impact on patients' quality of life. Read More

View Article and Full-Text PDF
October 2021

Neuroendocrine neoplasia and bone (Review).

Exp Ther Med 2021 Nov 26;22(5):1219. Epub 2021 Aug 26.

Department of Endocrinology, 'I. Hatieganu' University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania.

This is a narrative review focusing on neuroendocrine neoplasia (NEN) and bone status, in terms of metastases and osteoporosis/fractures. One fifth of NEN have skeletal dissemination, this affinity being regulated by intrinsic tumor factors such as the C-X-C chemokine receptor 4 (CXCR4). Bone colonization impairs the patient quality of life, representing a surrogate of reduced survival. Read More

View Article and Full-Text PDF
November 2021

Interventional Strategies in Cancer-induced Cardiovascular Disease.

Curr Oncol Rep 2021 09 27;23(11):133. Epub 2021 Sep 27.

Department of Cardiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Unit 1451, Houston, TX, 77030, USA.

Purpose Of Review: To highlight the range of illnesses and procedures that the interventional onco-cardiologists face in their daily practice, along with the recent additions to anti-cancer therapies and their related cardiotoxicity.

Recent Findings: Immune checkpoint inhibitors (ICI) are not devoid of cardiotoxicity as thought earlier and lead to an increased incidence of myocarditis. Transcatheter valve replacement has been shown to be a safer alternative to surgical replacement in cancer patients. Read More

View Article and Full-Text PDF
September 2021

Outcomes and periprocedural management of cardiac implantable electronic devices in patients with carcinoid heart disease.

Heart Rhythm 2021 12 21;18(12):2094-2100. Epub 2021 Aug 21.

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

Background: Carcinoid heart disease (CHD) is a rare complication of hormonally active neuroendocrine tumors that often requires surgical intervention. Data on cardiac implantable electronic device (CIED) implantation in patients with CHD are limited.

Objective: The purpose of this study was to evaluate the experience of CIED implantation in patients with CHD. Read More

View Article and Full-Text PDF
December 2021

A Systematic Review of Economic and Quality-of-Life Research in Carcinoid Syndrome.

Pharmacoeconomics 2021 11 11;39(11):1271-1297. Epub 2021 Aug 11.

Section of Cancer Economics and Policy, Department of Health Services Research, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Unit 1444, Houston, TX, 77030, USA.

Background: To date, the economic burden and patient-reported outcomes associated with carcinoid syndrome (CS) in patients with neuroendocrine tumor (NET) remain largely unknown.

Objectives: The objective of this study was to perform a systematic review of economic and quality-of-life (QOL) studies related to the treatment of CS.

Methods: Articles included in the review were extracted from PubMed, Embase, and the Cochrane Library. Read More

View Article and Full-Text PDF
November 2021

Clinical Features and Prognosis of Patients with Carcinoid Syndrome and Carcinoid Heart Disease: A Retrospective Multicentric Study of 276 Patients.

Neuroendocrinology 2022 26;112(6):547-554. Epub 2021 Jul 26.

ENETS Center of Excellence Bad Berka, Internal Medicine/Gastroenterology and Endocrinology, Bad Berka, Germany.

Introduction: Carcinoid syndrome is the most frequent functional syndrome of neuroendocrine neoplasia. It is characterized by flushing, diarrhea, wheezing, hypotension, and exanthema and may cause carcinoid heart disease.

Methods: We assessed clinical characteristics and prognosis of patients with carcinoid syndrome and carcinoid heart disease in 276 patients from 3 referral centers. Read More

View Article and Full-Text PDF

Multicenter, Observational Study of Lanreotide Autogel for the Treatment of Patients with Neuroendocrine Tumors in Routine Clinical Practice in Germany and Austria.

Exp Clin Endocrinol Diabetes 2021 Jul 22;129(7):500-509. Epub 2021 Jul 22.

ENDOC Center for Endocrine Tumors, Hamburg, Germany.

Background: The long-acting somatostatin analog lanreotide autogel is effective in the treatment of patients with neuroendocrine tumors.

Objective: To evaluate the long-term treatment response in patients with neuroendocrine tumors receiving lanreotide autogel in routine clinical practice.

Methods: Non-interventional, 24-month study in patients with neuroendocrine tumors treated with lanreotide autogel (NCT01840449). Read More

View Article and Full-Text PDF

A prospective study of carcinoid crisis with no perioperative octreotide.

Surgery 2022 01 3;171(1):88-93. Epub 2021 Jul 3.

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

Background: Carcinoid crises, defined as the sudden onset of hemodynamic instability in patients with neuroendocrine tumors undergoing operation, are associated with significantly increased risk of postoperative complications. Octreotide has been used prophylactically to reduce crisis rates as well as therapeutically to treat crises that still occur. However, studies using octreotide still report crisis rates of 3. Read More

View Article and Full-Text PDF
January 2022

Serotoninergic brain dysfunction in neuroendocrine tumor patients: A scoping review.

Compr Psychiatry 2021 08 19;109:152244. Epub 2021 May 19.

Department of Clinical and Biological Sciences, University of Turin, Orbassano (TO), Regione Gonzole, 10, 10043 Orbassano, Turin, Italy.

Introduction: Neuroendocrine tumors (NETs) are rare and malignant neoplasms characterized by their potential to produce metabolically active substances with the capacity to bring about clinical syndromes. The clinical expression of serotonin-producing NETs is known as carcinoid syndrome (CS). The synthesis of serotonin in the brain is dependent on tryptophan availability. Read More

View Article and Full-Text PDF

Use of healthcare REsources and associated COsts in controlled versus uncontrolled carcinoid SYndrome in patients with neuroendocrine tumours: the RECOSY study.

Clin Transl Oncol 2021 Oct 9;23(10):2046-2056. Epub 2021 Jun 9.

Endocrinology Service, Hospital Bellvitge, Hospitalet de Llobregat, Barcelona, Spain.

Purpose: To report healthcare resource use and associated costs in controlled versus uncontrolled carcinoid syndrome (CS) in patients with neuroendocrine tumours.

Methods: A cross-sectional, non-interventional multicentre study was conducted with retrospective data analysis. Resource use was compared between two patient groups: those with controlled CS (> 12 months with no uncontrolled CS episodes) and uncontrolled CS (< 12 months since last uncontrolled episode). Read More

View Article and Full-Text PDF
October 2021

Peptide Receptor Radionuclide Therapy With 177Lu-DOTATATE for Symptomatic Control of Refractory Carcinoid Syndrome.

J Clin Endocrinol Metab 2021 08;106(9):e3665-e3672

Department of Internal Medicine, Sector Endocrinology, ENETS Center of Excellence, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands.

Context: Peptide receptor radionuclide therapy (PRRT) with [Lutetium-177-DOTA0-Tyr3]octreotate (177Lu-DOTATATE) results in an increase of progression-free survival and quality of life in patients with progressive, well-differentiated neuroendocrine neoplasms (NENs).

Objective: To study the effect of 177Lu-DOTATATE in patients with carcinoid syndrome and radiologically stable or newly diagnosed disease treated solely for the purpose of symptom reduction.

Design: Retrospective cohort study. Read More

View Article and Full-Text PDF

Long-Term Treatment with Telotristat Ethyl in Patients with Carcinoid Syndrome Symptoms: Results from the TELEPATH Study.

Neuroendocrinology 2022 3;112(3):298-310. Epub 2021 May 3.

Section of Hematology/Oncology, Boston University and Boston Medical Center, Boston, Massachusetts, USA.

Introduction: Telotristat ethyl is indicated for use in combination with somatostatin analogs (SSAs) to treat carcinoid syndrome (CS) diarrhea uncontrolled by SSAs alone in adults, but long-term safety and efficacy data beyond 48 weeks are needed.

Objectives: The aims of the study were to evaluate the long-term safety and tolerability of telotristat ethyl and its effect on quality of life (QOL) in patients with CS.

Methods: In this phase 3, nonrandomized, multicenter, open-label, long-term extension study (TELEPATH), patients who participated in phase 2 or 3 trials of telotristat ethyl continued treatment at their present dose level (250 or 500 mg thrice daily) for 84 weeks. Read More

View Article and Full-Text PDF

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

View Article and Full-Text PDF