偶然发现心脏副神经节瘤。

Q4 Medicine

JACC. Case reports Pub Date : 2025-09-10 DOI:10.1016/j.jaccas.2025.104998

Kishan Srikanth MD , Kyung Min MD , Tann Lien DO , Steven Colicci RT (R) (MR) (MRSO) , Amber Wheeler MD , Salvador Hernandez MD , Jennifer Ting MD

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引用次数: 0

摘要

背景：副神经节瘤是一种罕见的神经内分泌肿瘤，起源于自主神经系统的神经嵴细胞系。近年来这些肿瘤的检测增加可归因于成像技术的进步。病例总结：一名59岁男性，进行常规的初级保健评估，偶然发现有2/6舒张期杂音。超声心动图和心脏磁共振显示右心室房室沟内有一个大而有边界的肿块。冠状动脉造影显示该肿块高度血管化，由右冠状动脉供应。血清学检查显示血清肾上腺素和尿多巴胺水平明显升高。dotatate -正电子发射断层扫描/计算机断层扫描证实了心脏副神经节瘤的诊断。讨论：由于儿茶酚胺的分泌，心脏副神经节瘤与显著的发病率相关，并且很少有恶性潜能。心脏副神经节瘤也可无症状，使诊断具有挑战性。鉴于其罕见性，缺乏关于这些肿瘤的诊断和管理策略的循证指南。关键信息：心脏副神经节瘤的诊断需要血清学和影像学检查的结合。多学科评价对于制定有效的管理策略至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Cardiac Paraganglioma Discovered Incidentally

Background

Paragangliomas are rare neuroendocrine tumors, arising from neural crest cell lines in the autonomic system. The increased detection of these tumors in recent years can be attributed to advances in imaging techniques.

Case Summary

A 59-year-old man, presenting for a routine primary care evaluation, was incidentally noted to have a 2/6 diastolic murmur. Echocardiography and cardiac magnetic resonance characterized a large, circumscribed mass within the atrioventricular groove of the right ventricle. Coronary angiography demonstrated that this mass was highly vascularized, supplied by the right coronary artery. Serologic work-up revealed significantly elevated serum epinephrine and urinary dopamine levels. DOTATATE-positron emission tomography/computed tomography confirmed the diagnosis of a cardiac paraganglioma.

Discussion

Cardiac paragangliomas are associated with significant morbidity due to catecholamine secretion and rarely malignant potential. Cardiac paragangliomas can also be asymptomatic, making diagnosis challenging. Given their rarity, there is a paucity of evidence-based guidelines regarding diagnostic and management strategies for these tumors.

Take-Home Messages

Cardiac paragangliomas require a combination of serological and imaging tests for diagnosis. Multidisciplinary evaluation is essential for developing effective management strategies.

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