Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report. Issue 3 (17th April 2020)

• Record Type: Journal Article
• Authors: Trêpa, Maria; Silveira, Inês; Amaral, Cláudia; Luz, André
• Editors: Kunadian, Vijay; Akodad, Mariama; Deharo, Pierre; Søholm, Helle; Farag, Mohamed F; Vamvakidou, Anastasia; Ranganathan, Deepti
• Is Part Of: European heart journal. Volume 4:Issue 3(2020)
• Page Start: 1
• Page End: 6
• Publication Date: 2020-04-17
• Subjects: Mediastinal paraganglioma -- Coronary embolization -- Radionuclide therapy -- Neuroendocrine tumour -- Case report; Cardiology -- Periodicals; Cardiology -- Case studies -- Periodicals; Heart -- Diseases -- Periodicals; Heart -- Diseases -- Case studies -- Periodicals; 616.12
• DOI: 10.1093/ehjcr/ytaa062
• Languages: English
• ISSNs: 2514-2119

Abstract

Abstract: 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.