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Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report

BACKGROUND: Mediastinal paragangliomas (PGs) are rare and particularly challenging neuroendocrine tumours. Clinical presentation is heterogeneous and tumour resection can be challenging due to bleeding and the risk of catecholamine surges in functional tumours. CASE SUMMARY: A 36-year-old man with m...

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Autores principales: Trêpa, Maria, Silveira, Inês, Amaral, Cláudia, Luz, André
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319854/
https://www.ncbi.nlm.nih.gov/pubmed/32617509
http://dx.doi.org/10.1093/ehjcr/ytaa062
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author Trêpa, Maria
Silveira, Inês
Amaral, Cláudia
Luz, André
author_facet Trêpa, Maria
Silveira, Inês
Amaral, Cláudia
Luz, André
author_sort Trêpa, Maria
collection PubMed
description BACKGROUND: Mediastinal paragangliomas (PGs) are rare and particularly challenging neuroendocrine tumours. Clinical presentation is heterogeneous and tumour resection can be challenging due to bleeding and the risk of catecholamine surges in functional tumours. CASE SUMMARY: A 36-year-old man with multiple cardiovascular risk factors was admitted with subacute heart failure. Investigations revealed a large non-metastatic functional mediastinal PG irrigated mainly by a left circumflex coronary anomalous feeder branch. The surgical risk was deemed very high due to patient comorbidities, tumour vascularization, and close relation to major thoracic structures. A multidisciplinary team decided to perform embolization of the anomalous coronary branch followed by peptide-receptor radionuclide therapy with 177-LuDOTATE aiming to decrease tumour size and perioperative risk. Follow-up studies showed a reduction in tumour vascularization, size, and hormonal production. DISCUSSION: The innovative strategy of combining embolization of the anomalous feeder branch with radionuclide therapy proved to be a promising approach.
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spelling pubmed-73198542020-07-01 Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report Trêpa, Maria Silveira, Inês Amaral, Cláudia Luz, André Eur Heart J Case Rep Case Reports BACKGROUND: Mediastinal paragangliomas (PGs) are rare and particularly challenging neuroendocrine tumours. Clinical presentation is heterogeneous and tumour resection can be challenging due to bleeding and the risk of catecholamine surges in functional tumours. CASE SUMMARY: A 36-year-old man with multiple cardiovascular risk factors was admitted with subacute heart failure. Investigations revealed a large non-metastatic functional mediastinal PG irrigated mainly by a left circumflex coronary anomalous feeder branch. The surgical risk was deemed very high due to patient comorbidities, tumour vascularization, and close relation to major thoracic structures. A multidisciplinary team decided to perform embolization of the anomalous coronary branch followed by peptide-receptor radionuclide therapy with 177-LuDOTATE aiming to decrease tumour size and perioperative risk. Follow-up studies showed a reduction in tumour vascularization, size, and hormonal production. DISCUSSION: The innovative strategy of combining embolization of the anomalous feeder branch with radionuclide therapy proved to be a promising approach. Oxford University Press 2020-04-17 /pmc/articles/PMC7319854/ /pubmed/32617509 http://dx.doi.org/10.1093/ehjcr/ytaa062 Text en © The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Reports
Trêpa, Maria
Silveira, Inês
Amaral, Cláudia
Luz, André
Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title_full Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title_fullStr Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title_full_unstemmed Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title_short Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
title_sort innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319854/
https://www.ncbi.nlm.nih.gov/pubmed/32617509
http://dx.doi.org/10.1093/ehjcr/ytaa062
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