scholarly journals Carcinoid Syndrome and Carcinoid Heart Disease as Manifestations of Non-Metastatic Ovarian Neuroendocrine Tumour

2017 ◽  
Vol 30 (5) ◽  
pp. 421 ◽  
Author(s):  
Joana Simões-Pereira ◽  
Lai Mun Wang ◽  
Attila Kardos ◽  
Ashley Grossman
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Early Recognition ◽  
Tumour Resection ◽  
Carcinoid Heart Disease ◽  
Additional Burden ◽  
Gastrointestinal Neuroendocrine Tumours ◽  
Surgical Valve Replacement

The carcinoid syndrome is rare but it is associated with carcinoid heart disease in more than a half of the cases. Carcinoid heart disease is typically characterised by morphological and functional modifications of right-sided valves. Its aetiology is probable multifactorial but serotonin appears to play a key role in the development of this valvular disease. Unlike gastrointestinal neuroendocrine tumours, ovarian neuroendocrine tumours can present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases; such ovarian neuroendocrine tumours are a unique clinical entity. The additional burden of cardiac impairment in these patients represents a significant reduction in survival. Early recognition and surgical valve replacement before advanced heart failure is established may improve the clinical outcome. We report the case of a woman with an ovarian neuroendocrine tumour and highly symptomatic carcinoid heart disease who was submitted to tumour resection followed by valvuloplasty. She demonstrated an outstanding clinical improvement and has remained free of tumour and symptomatology.

Treatment of metastatic midgut neuroendocrine tumour associated with carcinoid syndrome

2014 ◽  
Vol 155 (5) ◽  
pp. 194-198
Author(s):  
Andrea Uhlyarik ◽  
Erika Lahm ◽  
József Vachaja ◽  
Zsuzsanna Pápai
Keyword(s):  
Metastatic Disease ◽  
Case History ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Somatostatin Receptor ◽  
Tumour Response ◽  
Tumour Resection ◽  
Peptide Receptor ◽  
Long Acting

Although the incidence of neuroendocrine tumours is low, their prevalence is high due to the usually slow course of the disease. Between July 1, 2008 and July 1, 2013 the authors evaluated 56 patients with well-differentiated or moderately differentiated neuroendocrine tumours; 36 patients with metastatic disease underwent treatment while 17 patients who had tumour resection were followed without additional treatment. All patients with metastatic disease received long acting octreotide, and additional therapy was based on the site of origin, grade of differentiation, Ki67 index, and focal labelling of the tumours during somatostatin-receptor or metaiodo-benzyl-guanidine scintigraphy. The authors present a detailed case history of a patient with carcinoid syndrome due to a metastatic midgut neuroendocrine tumour, who received long acting octreotide and peptide receptor radionuclide treatment. In this patient an objective tumour response was reached in addition to the resolution of symptoms of carcinoid syndrome. The authors conclude that the case history confirms previous observations showing that long acting octreotide combined with peptide receptor radionuclide treatment may provide long survival with good quality of life in a patient with metastatic midgut neuroendocrine tumour accompanied with carcinoid syndrome. Orv. Hetil., 2014, 155(5), 194–198.

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

Analysis of association between carcinoid heart disease and mesenteric fibrosis in patients with midgut carcinoid tumour

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e15650-e15650
Author(s):  
K. Desai ◽  
S. Bhattacharya ◽  
R. Srirajaskanthan ◽  
L. Morgan-Rowe ◽  
C. Toumpanakis ◽  
...  
Keyword(s):  
Heart Disease ◽  
Small Bowel ◽  
Bowel Obstruction ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Morbidity And Mortality ◽  
Carcinoid Heart Disease ◽  
Cardiovascular Assessment ◽  
Relationship Of ◽  
The Relationship

e15650 Background: Valvular heart disease in patients with carcinoid syndrome is due to development of fibrosis and endocardial thickening in the heart. The most accepted theory relating to development of heart disease is secretion of serotonin (5- hydroxy tryptamine) by the tumour or metastases. Mesenteric fibrosis develops as a result of peptides e.g.TGF beta released by local metastases. Both complications cause significant morbidity and mortality in patients with carcinoid syndrome. Aim: to evaluate the relationship of carcinoid heart disease and mesenteric fibrosis. Methods: We analysed retrospectively all patients (n=200) who had metastatic (liver metatstasis) neuroendocrine tumour and carcinoid syndrome with the primary clearly identified within the small bowel or caecum. We aimed to evaluate clinically symptoms of mesenteric ischemia and small bowel obstruction and also radiological presence of desmoplasia and small bowel dilatation. All patients underwent clinical and echocardiographic cardiovascular assessment to determine the presence of carcinoid heart disease. Results: Of 200 patients 42 had echocardiographic evidence of carcinoid heart disease. 4 patients had evidence of both mesenteric desmoplasia and carcinoid heart disease. 38 patients had carcinoid heart disease alone. Of the remaining 158 patients who had no evidence of carcinoid heart disease, 24 of them had evidence of desmoplasia and/or clinical evidence of sub-acute bowel obstruction where no other cause could be found. The remaining 134 patients had none of these complications. Comparing the relationship of Carcinoid heart disease and mesenteric fibrosis using two tailed exact Fisher's test, p value is 0.45, demonstrating they are statistically independent. We are currently assessing the effect on mortality. Conclusions: Carcinoid heart disease and mesenteric fibrosis (and its effects) are important determinants in morbidity and mortality of patients with midgut neuroendocrine tumour. Although carcinoid heart disease and mesenteric fibrosis are part of the same patho-physiologic process affecting patients with midgut neuroendocrine tumour, the results suggest that progression of these complications is independent of each other. No significant financial relationships to disclose.

Evaluation of Clinical Prognostic Factors and Further Delineation of the Effect of Mesenteric Fibrosis on Survival in Advanced Midgut Neuroendocrine Tumours

2018 ◽  
Vol 107 (3) ◽  
pp. 292-304 ◽  
Author(s):  
Faidon-Marios Laskaratos ◽  
Martin Walker ◽  
Dominic Wilkins ◽  
Alexander Tuck ◽  
Shashank Ramakrishnan ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Large Scale ◽  
Neuroendocrine Tumours ◽  
Primary Tumour ◽  
Stage Iv ◽  
Epidemiological Data ◽  
Tumour Resection ◽  
Carcinoid Heart Disease ◽  
Factors Affecting ◽  
Stage Iv Disease

Background: Small intestinal neuroendocrine tumours (SI NETs) represent 30–50% of small bowel neoplasms and often present at an advanced stage. To date, there is relatively limited literature regarding prognostic factors affecting overall survival (OS) in stage IV disease. In addition, the prevalence of mesenteric fibrosis (MF) in SI NETs and its effect on OS have not been sufficiently explored in the literature. Aim: The primary aim of this study was to perform a large-scale survival analysis in an institutional cohort of 387 patients with metastatic (stage IV) SI NETs. The secondary aim was to provide epidemiological information regarding the prevalence of MF and to evaluate its effect on OS. Results: The median OS was 101 months (95% CI 84, 118). Age > 65 years, mesenteric metastases with and without desmoplasia, liver metastases, carcinoid heart disease (CHD) and bone metastases were associated with a significantly shorter OS, while primary tumour resection was predictive of a longer OS. The benefit of surgical resection was limited to symptomatic patients. MF was present in approximately 50% of patients with mesenteric lymphadenopathy. Elevated urinary 5-HIAA levels correlated strongly with the presence of CHD (p < 0.001) and to a lesser extent (p = 0.02) with MF. MF and CHD did not usually co-exist, suggesting that different mechanisms are likely to be involved in the development of these fibrotic complications. Conclusions: This study has identified specific prognostic factors in a large cohort of 387 patients with advanced SI NETs and has provided useful epidemiological data regarding carcinoid-related fibrotic complications.

Positional changes leading to transient ischaemia in a patient with neuroendocrine carcinoma of the lung

2021 ◽  
Vol 14 (6) ◽  
pp. e241674
Author(s):  
James Livesay ◽  
Hassan Tahir ◽  
Raj Baljepally
Keyword(s):  
Heart Disease ◽  
Neuroendocrine Carcinoma ◽  
Rare Case ◽  
Neuroendocrine Tumours ◽  
Body Position ◽  
Coronary Artery Spasm ◽  
Coronary Spasm ◽  
Carcinoid Heart Disease ◽  
St Elevation ◽  
Early Manifestation

Coronary spasm in carcinoid heart disease has an incidence of 10%, but is rare in patients with neuroendocrine tumours without carcinoid heart disease. We present a rare case of right coronary artery spasm and ST elevations secondary to a neuroendocrine carcinoma of the lung, uniquely provoked by positional changes. Our patient is a 55-year-old man with recurrent ST-elevation myocardial infarction secondary to coronary vasospasm that was diagnosed with neuroendocrine carcinoma of the lung. We believe his positional coronary spasm episodes were likely due to intermittent tumour compression from changes in body position. Our case highlights positional coronary spasm as a previously unreported and potentially early manifestation of neuroendocrine carcinoma of the lung.

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 P55 Carcinoid syndrome on etanercept and subsequent treatment with secukinumab in a patient with psoriatic arthritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Ali S. F Sheikh ◽  
Sagar G Srivastva ◽  
Fiona Wood
Keyword(s):  
Psoriatic Arthritis ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Early Recognition ◽  
Carcinoid Tumour ◽  
Safety Data ◽  
Biologic Agents ◽  
Tnf Alpha ◽  
First Line ◽  
Alpha Blockers

Abstract Background Psoriatic arthritis requires early recognition and treatment for prevention of disease progression. Conventional disease modifying drugs are first-line agents followed by biologic DMARDs for patients with active disease. TNF-alpha blockers are first line biologic agents in the UK. Th17 inhibitors are used since the elucidation of Th17 pathway. Safety and efficacy profiles of biologic agents inhibiting the Th17 pathway, including secukinumab (IL-17A) and ustekinumab (IL-12/23p40) have been studied. Methods We report a case of carcinoid syndrome in a lady on etanercept for psoriatic arthritis, carcinoid as a potential TNF alpha side effect. We also report safety of Th17 (secukinumab) inhibitors in the patient to date (&gt;1 year). Results A female with a history of acne rosacea, was diagnosed with psoriatic arthritis in 2000 age 32. Initial sulphasalazine failed, then received methotrexate until 2011, when her arthritis flared. She was commenced on etanercept which proved effective. After 4 years of etanercept and methotrexate, her liver profile became deranged. Investigations off treatment included ultrasound abdomen demonstrating a liver mass, which resembled focal nodular hyperplasia on magnetic resonance imaging. Further screening revealed high urinary 5HIAA (527 umol/24h) and raised chromogranin A & B levels (1574 pmol/L and 373 pmol/L respectively). She had no symptoms suggestive of carcinoid, although facial flushing could have been camouflaged by her rosacea. Octreotide scan was positive, CT enterogram showed a distal ileal neuroendocrine tumour with adjacent lymphadenopathy. She underwent right hemihepatectomy and hemicolectomy. Her liver profile deteriorated again on methotrexate, leflunomide was ineffective. In November 2017 she was started on ustekinumab, which was ineffective and was withdrawn after 8 months. There was no alteration in carcinoid blood markers and no CT changes. In July 2018, she was commenced on secukinumab, which has allowed reduction in steroids. The patient is aware of lack of safety data in her circumstances. Regular surveillance has shown no recurrence of carcinoid with serial negative 5HIAA and chromogranin levels. There are no progressive CT changes at 1 year. Conclusion This is a case of carcinoid tumour occurring on TNF-alpha blockers and may represent a rare complication. Screening biomarkers including 5HIAA and chromogranin levels can be useful if disease is suspected. We could not find other similar case reports to guide further management. Within time limited data available - ustekinumab had no effect on the carcinoid. Th17 inhibitors can be safe options for treating psoriatic arthritis and psoriasis with highly sustained efficacy and favourable safety profile seen in large clinical trials. In this case, after &gt; one year of secukinumab treatment - there is no adverse effect on carcinoid syndrome. Disclosures A.S.F. Sheikh None. S.G. Srivastva None. F. Wood None.

scholarly journals Intraoperative carcinoid syndrome during small-bowel neuroendocrine tumour surgery

2018 ◽  
Vol 7 (12) ◽  
pp. 1245-1250 ◽  
Author(s):  
Myrtille Fouché ◽  
Yves Bouffard ◽  
Mary-Charlotte Le Goff ◽  
Johanne Prothet ◽  
François Malavieille ◽  
...  
Keyword(s):  
Risk Factors ◽  
Small Bowel ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Anaesthetic Management ◽  
Primary Objective ◽  
Carcinoid Heart Disease ◽  
Continuous Administration ◽  
Arterial Blood ◽  
Carcinoid Crisis

Only few descriptions of intraoperative carcinoid syndrome (ioCS) have been reported. The primary objective of this study was to describe ioCS. A second aim was to identify risk factors of ioCS. We retrospectively analysed patients operated for small-bowel neuroendocrine tumour in our institution between 2007 and 2015, and receiving our preventive local regimen of octreotide continuous administration. ioCS was defined as highly probable in case of rapid (<5 min) arterial blood pressure changes ≥40%, not explained by surgical/anaesthetic management and regressive ≥20% after octreotide bolus injection. Probable cases were ioCS which did not meet all criteria of highly-probable ioCS. Suspected ioCS were detected on the anaesthesia record by an injection of octreotide due to a manifestation which did not meet the criteria for highly-probable or probable ioCS. A total of 81 patients (liver metastases: 59, prior carcinoid syndrome: 49, carcinoid heart disease: 7) were included; 139 ioCS occurred in 45 patients: 45 highly probable, 67 probable and 27 suspected. ioCs was hypertensive (91%) and/or hypotensive (29%). There was no factor, including the use of vasopressors, significantly associated with the occurrence of an ioCS. All surgeries were completed and one patient died from cardiac failure 4 days after surgery. After preoperative octreotide continuous infusion, ioCS were mainly hypertensive. No ioCS risk factors, including vasopressor use, were identified. No intraoperative carcinoid crisis occurred, suggesting the clinical relevance of a standardized octreotide prophylaxis protocol.

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.

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