Chromogranin A (CgA) as a biomarker in carcinoid heart disease and NETG1/G2 neuroendocrine neoplasms of the small intestine (SI-NENs) related carcinoid syndrome

2021 ◽  
Author(s):  
Sonia J. Konsek-Komorowska ◽  
Mariola Pęczkowska ◽  
Agnieszka D. Kolasińska-Ćwikła ◽  
Marek Konka ◽  
Eryk Chrapowicki ◽  
...  
Keyword(s):  
Small Intestine ◽  
Heart Disease ◽  
Chromogranin A ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Neoplasms ◽  
Carcinoid Heart Disease

scholarly journals Multimodality imaging in carcinoid heart disease

Open Heart ◽  
2019 ◽  
Vol 6 (1) ◽  
pp. e001060 ◽  
Author(s):  
Ali M Agha ◽  
Juan Lopez-Mattei ◽  
Teodora Donisan ◽  
Dinu Balanescu ◽  
Cezar A Iliescu ◽  
...  
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Multimodality Imaging ◽  
Imaging Techniques ◽  
Neuroendocrine Neoplasms ◽  
Carcinoid Heart Disease ◽  
Imaging Studies ◽  
Diagnosis And Prognosis ◽  
Positron Emission ◽  
Integral Role

Neuroendocrine neoplasms arise from the gastrointestinal tract and can lead to carcinoid syndrome. Carcinoid heart disease affects more than half of these patients and is the initial presentation of carcinoid syndrome in up to 20 % of patients. Carcinoid heart disease typically leads to valve dysfunction, but in rare instances, carcinoid tumours can also metastasise to the endocardium and myocardium. Cardiovascular imaging plays an integral role in the diagnosis and prognosis of carcinoid heart disease. The use of multimodality imaging techniques including echocardiography, cardiac MRI, cardiovascular CT and positron emission tomography have allowed for a more comprehensive assessment of carcinoid heart disease. In this review, we discuss the features of carcinoid heart disease observed on multimodality imaging, indications for obtaining imaging studies and their role in carcinoid heart disease management.

scholarly journals Biomarkers in Small Intestine NETs and Carcinoid Heart Disease: A Comprehensive Review

Biology ◽  
2021 ◽  
Vol 10 (10) ◽  
pp. 950
Author(s):  
Markos Kalligeros ◽  
Leonidas Diamantopoulos ◽  
Christos Toumpanakis
Keyword(s):  
Small Intestine ◽  
Heart Disease ◽  
Chromogranin A ◽  
Clinical Utility ◽  
Disease Status ◽  
Carcinoid Heart Disease ◽  
Mrna Transcript ◽  
Comprehensive Review ◽  
Diagnosis And Management ◽  
Novel Biomarkers

Biomarkers remain a valuable tool for the diagnosis and management of Neuroendocrine tumors (NETs). Traditional monoanalyte biomarkers such as Chromogranin A (CgA) and 5-Hydrocyondoleacetic acid (5-HIAA) have been widely used for many years as diagnostic, predictive and prognostic biomarkers in the field of NETs. However, the clinical utility of these molecules often has limitations, mainly inherent to the heterogeneity of NETs and the fact that these tumors can often be non-secretory. The development of new molecular multianalyte biomarkers, especially the mRNA transcript based “NETest”, has rapidly evolve the field and gives the ability for a “liquid biopsy” which can reliably assess disease status in real time. In this review we discuss the use of established and novel biomarkers in the diagnosis and management of small intestine NETs and carcinoid heart disease.

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

2020 ◽  
Author(s):  
Ali J Ebrahimi ◽  
Peter Marogil ◽  
Gregory Von Mering ◽  
Mustafa Ahmed
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Three Dimensional ◽  
Systemic Circulation ◽  
Neuroendocrine Neoplasms ◽  
Carcinoid Heart Disease ◽  
Malignant Carcinoid ◽  
Vasoactive Substances ◽  
Malignant Carcinoid Syndrome ◽  
Echocardiographic Images

Abstract Background Carcinoid syndrome is a rare disease caused by malignant neuroendocrine neoplasms. When vasoactive substances enter the systemic circulation, the triad of cutaneous flushing, bronchospasm, and diarrhoea often characterize carcinoid syndrome. Rarely, carcinoid syndrome can progress to involve the cardiac system, a condition known as carcinoid heart disease, often affecting right-sided valvular structures. Case summary Here, we present a case of malignant carcinoid syndrome with associated carcinoid heart disease in a 63-year-old female. The diagnosis of her dual regurgitant and stenotic valvular disease is detailed, with accompanying two- and three-dimensional echocardiographic images demonstrating the patient’s complex tricuspid dysfunction. Discussion Carcinoid heart disease encompasses a rare but important subset of valvular dysfunction caused by circulating vasoactive substances. Diagnosis utilizing serum studies, computed tomography scans, and echocardiography can help expedite the diagnosis and treatment of such rare conditions, and assist in the avoidance of complications. Despite its relatively well-recognized clinical presentation, carcinoid syndrome and its associated heart disease still remains a challenging condition to manage and treat, often requiring the input of several subspecialties to treat the condition appropriately.

scholarly journals Carcinoid heart disease

2013 ◽  
Vol 154 (14) ◽  
pp. 546-550
Author(s):  
Ágnes Bencze ◽  
Nikolette Szücs ◽  
Péter Igaz ◽  
Katalin Leiszter ◽  
Zsolt Nagy ◽  
...  
Keyword(s):  
Heart Disease ◽  
Life Expectancy ◽  
Case History ◽  
Carcinoid Syndrome ◽  
Rare Complication ◽  
Neuroendocrine Cells ◽  
Biologically Active ◽  
Therapeutic Options ◽  
Carcinoid Heart Disease ◽  
History Of

Carcinoids are rare tumors originating from neuroendocrine cells. A large proportion of these tumors produce serotonin and other biologically active hormones which may produce carcinoid syndrome characterized by flushing, diarrhoea and bronchospasm. Carcinoid heart disease, a rare complication of carcinoid syndrome, may itself have a great impact on life expectancy of patients with carcinoid syndrome. The authors present a case history of a patients with carcinoid heart disease and they review the symptoms, diagnosis and therapeutic options of this rare complication of carcinoid syndrome. Orv. Hetil., 2013, 154, 546–550.

Ovarian metastasis from midgut neuroendocrine tumors: Incidence, clinical implications, and management options—An update.

2017 ◽  
Vol 35 (4_suppl) ◽  
pp. 446-446 ◽  
Author(s):  
Aman Chauhan ◽  
Lowell Brian Anthony ◽  
David T Beyer ◽  
Yi-Zarn Wang
Keyword(s):  
Heart Disease ◽  
Neuroendocrine Tumors ◽  
Carcinoid Syndrome ◽  
Survival Rates ◽  
Ovarian Metastasis ◽  
Clinical Implications ◽  
Carcinoid Heart Disease ◽  
Bilateral Oophorectomy ◽  
Female Patients ◽  
Midgut Net

446 Background: Midgut neuroendocrine tumors (NETs) are rare malignancies that can produce carcinoid syndrome and/or carcinoid heart disease. The incidence of ovarian metastasis, their clinical implications, and the optimal management strategy for these metastasis have not been well studied. We hypothesized that patients with ovarian metastasis will have a high incidence of carcinoid syndrome and carcinoid heart disease. Methods: Charts of 431 female patients seen in our NET clinic between October 2006 and December 2014 with a diagnosis of midgut NET were reviewed. Patients who had a previous bilateral oophorectomy with or without total abdominal hysterectomy were excluded. The incidence of ovarian metastasis, carcinoid syndrome and carcinoid heart disease was calculated. Median survival and 5-year survival rates were determined via the Kaplan-Meier Method. Results: Forty-one (41/431, 10%) patients were found to have ovarian metastasis. Twenty-four (24/41, 59%) patients exhibited carcinoid syndrome and three (3/24, 13%) patients with carcinoid syndrome developed carcinoid-related heart disease. 5-year survival rates for the patients with and without ovarian metastasis were 92% and 87%, respectively. Conclusions: Ovarian metastasis was found in 10% patients with metastatic midgut NET. Fifty-nine percent of these patients exhibited carcinoid syndrome and thirteen percent of which developed carcinoid heart disease, in spite of aggressive somatostatin analog therapy. To improve quality of life, routine therapeutic or prophylactic oophorectomy is recommended for female patients undergoing cytoreductive surgery for their midgut NET, especially for those who are peri- or post-menopausal.

The optimal use of cardiac imaging in the quantification of carcinoid heart disease

2013 ◽  
Vol 20 (5) ◽  
pp. R247-R255 ◽  
Author(s):  
Rebecca Dobson ◽  
Daniel J Cuthbertson ◽  
Malcolm I Burgess
Keyword(s):  
Heart Disease ◽  
Cardiac Imaging ◽  
Carcinoid Syndrome ◽  
Imaging Modalities ◽  
Carcinoid Heart Disease ◽  
Important Complication ◽  
Main Technique ◽  
Severity Of The Disease

Carcinoid heart disease is a rare cause of right-sided valvular dysfunction, primarily mediated by serotonin. It is an important complication in patients with carcinoid syndrome and occurs in 20–50% of such patients. Echocardiography is the main technique used for the assessment of carcinoid heart disease, but other imaging modalities are also important, particularly in the quantification of the severity of the disease. We sought to review the role of cardiac imaging in the assessment of carcinoid heart disease.

scholarly journals Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-11 ◽  
Author(s):  
Dominique Clement ◽  
John Ramage ◽  
Raj Srirajaskanthan
Keyword(s):  
Growth Factor ◽  
Transforming Growth Factor Beta ◽  
Carcinoid Syndrome ◽  
Transforming Growth Factor ◽  
Tissue Growth ◽  
Care Plan ◽  
Prophylactic Surgery ◽  
Neuroendocrine Neoplasms ◽  
Carcinoid Heart Disease ◽  
Carcinoid Crisis

Carcinoid syndrome (CS) develops in patients with hormone-producing neuroendocrine neoplasms (NENs) when hormones reach a significant level in the systemic circulation. The classical symptoms of carcinoid syndrome are flushing, diarrhoea, abdominal pain, and wheezing. Neuroendocrine neoplasms can produce multiple hormones: 5-hydroxytryptamine (serotonin) is the most well-known one, but histamine, catecholamines, and brady/tachykinins are also released. Serotonin overproduction can lead to symptoms and also stimulates fibrosis formation which can result in development of carcinoid syndrome-associated complications such as carcinoid heart disease (CaHD) and mesenteric fibrosis. Transforming growth factor beta (TGF-β) is one of the main factors in developing fibrosis, but platelet-derived growth factor (PDGF), basic fibroblast growth factor (FGF2), and connective tissue growth factor (CTGF or CCN2) are also related to fibrosis development. Treatment of CS focuses on reducing serotonin levels with somatostatin analogues (SSA’s). Telotristat ethyl and peptide receptor radionuclide therapy (PRRT) have recently become available for patients with symptoms despite being established on SSA’s. Screening for CaHD is advised, and early intervention prolongs survival. Mesenteric fibrosis is often present and associated with poorer survival, but the role for prophylactic surgery of this is unclear. Depression, anxiety, and cognitive impairment are frequently present symptoms in patients with CS but not always part of their care plan. The role of antidepressants, mainly SSRIs, is debatable, but recent retrospective studies show evidence for safe use in patients with CS. Carcinoid crisis is a life-threatening complication of CS which can appear spontaneously but mostly described during surgery, anaesthesia, chemotherapy, PRRT, and radiological procedures and may be prevented by octreotide administration.

scholarly journals 1640 Pulmonary valve in carcinoid disease: be suspicious of functional assessment

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A L Gamarra Lobato ◽  
A M Rojas ◽  
A Cecconi ◽  
L Dominguez ◽  
A Benedicto ◽  
...  
Keyword(s):  
Heart Disease ◽  
Right Ventricle ◽  
Tricuspid Valve ◽  
Functional Assessment ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Previous History ◽  
Pulmonary Regurgitation ◽  
Lower Limbs ◽  
Carcinoid Heart Disease

Abstract Carcinoid heart disease occurs in 50% of patients with carcinoid syndrome, especially on serotonin-secreting neuroendocrine tumors (NET) [1, 2]. Carcinoid heart disease"s typical findings are carcinoid plaques, composed of smooth muscle, myofibroblasts and endothelium. These plaques, most frequently localized on the right side valvular cusps and leaflets, produce fixation and retraction, causing regurgitation and/or stenosis [3, 4, 5]. Left-sided heart disease is rare (<10%), as serotonin is purified in the pulmonary circulation [3, 6, 7]. We present the case of a 67 year old female patient with previous history of right ovarian NET. She presented with dyspnea (II-III NYHA functional class), and slight edema in lower limbs. She denied symptoms of carcinoid syndrome before or after the ovarian surgery. Examination showed an increased jugular venous pulse. Transthoracic echocardiography (TTE) showed severely dilated right cavities and a rigid, immobile tricuspid valve, with massive tricuspid regurgitation (Panel A, 1). Anatomy of pulmonary valve was not properly visualized but jet area of pulmonary regurgitation was small (Panel A, 2). Left cavities and valves were intact. As TTE was not anatomically conclusive, a cardiac magnetic resonance (CMR) and a cardiac computed tomography (CCT) were performed to assess the carcinoid involvement of pulmonary valve. CMR showed severely dilated right ventricle with mild impairment of systolic function (Panel A, 3). Based on phase contrast imaging, pulmonary regurgitation fraction was 14%, suggestive of a mild grade. However, CCT showed a diffuse thickening of the pulmonary valve, with complete opening during diastole (Panel A, 4, arrowhead). Finally, the patient underwent replacement of tricuspid valve and pulmonary valve for biological prosthesis without complications. Our case is remarkable because it highlights the limitations of the functional assessment of pulmonary regurgitation in the presence of a concomitant massive tricuspid valve regurgitation, since the rapid equalization of pressure between pulmonary artery and right ventricle reduces the expression of pulmonary regurgitation. For these reason, the anatomic assessment of the pulmonary valve is mandatory to stage the involvement of pulmonary valve in carcinoid disease. Abstract 1640 Figure. Panel A

scholarly journals P221 Carcinoid heart disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Tyminska ◽  
D Kaczmarska-Turek ◽  
A Kaplon-Cieslicka ◽  
J Kochanowski ◽  
P Scislo ◽  
...  
Keyword(s):  
Heart Disease ◽  
Tricuspid Regurgitation ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Good Control ◽  
Carcinoid Heart Disease ◽  
Long Acting ◽  
Surgical Valve Replacement

Abstract Carcinoid heart disease is a rare disease, which develops in 20-50% of patients with carcinoid syndrome and is a main predictor of clinical outcome in those patients. Typical cardiac involvement of this disease presents as primary diseases of the tricuspid or pulmonary valves with a rare affection of left sided heart valves. The characteristic pathological findings are endocardial plaques of fibrous which may involve not only the valve leaflets, but also the subvalvar apparatus.Typical management of patients with this condition consist of the treatment of right heart failure (HF), pharmacotherapy to reduce the secretion of tumour products, and surgical valve replacement. Here we report a 56-year old male admitted to the Department of Endocrinology because of flushing with abdominal pain and diarrhea 2-3 times a week for 2 years. During hospitalization carcinoid syndrome with metastases to the liver and abdominal lymph nodes was diagnosed. Treatment with a long-acting somatostatin analog was initiated, resulting in a good control of the symptoms of the carcinoid syndrome. Trans-thoracic echocardiography revealed right atrial and right ventricular enlargement with degenerative lesions of tricuspid valve leaflets and its subvalvular apparatus, with leaflet stiffening, retraction and malcoaptation, resulting in severe tricuspid regurgitation (vena contracta [VC] width - 7 mm). Pulmonic valve was also involved with thickening of pulmonary valve cusps leading to mild pulmonary stenosis (peak gradient [PG] - 27 mmHg, mean gradient [MG] - 14 mmHg) and mild pulmonary regurgitation. Moreover, signs of pulmonary hypertension (with tricuspid regurgitation pressure gradient [TRPG] of 50 mmHg and estimated systolic pulmonary artery pressure [SPAP] of 50-55 mmHg) was observed. There were no signs of hemodynamically significant left-sided valve disease, nor of any abnormalities in segmental or global left ventricular function. After 12 months of treatment with a long-acting somatostatin analog, the patient was reassessed. Despite a good control of carcinoid syndrome symptoms and reduction in carcinoid syndrome marker (5-hydroxyindoloacetic acid), exacerbates the tricuspid regurgitation and worsens right HF was observed. Control echocardiographic examination showed significant progression of the pulmonary valve disease with severe pulmonary regurgitation (VC width - 10 mm) and mild pulmonary stenosis (PG 18 mmHg, MG 8 mmHg), a deterioration of tricuspid regurgitation (VC width - 11 mm, effective regurgitant orifice area - 0.94 cm2, regurgitant volume - 64 ml) with further enlargement of the right ventricle and right atrium, and with a consequent decrease in TRPG value (25 mmHg), despite well preserved right ventricular systolic function (TAPSE - 24 mm). Due to disease progression, treatment of HF and peptide receptor radionuclide therapy were initiated. The patient was presented for surgical valve replacement, however he did not agree to surgical treatment. Abstract P221 Figure

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