Cutaia Aldo, Gaetani Clara, Fonio Paolo, Faletti Riccardo
Radiology Unit, Department of Surgical Sciences, University of Turin, Via Genova 3, 10126 Turin, Italy.
Eur Heart J Case Rep. 2023 Aug 1;7(8):ytad363. doi: 10.1093/ehjcr/ytad363. eCollection 2023 Aug.
Paragangliomas (PGLs) are rare neuroendocrine tumours that originate from extra-adrenal location. Cardiac PGLs can cause severe hypertension, palpitations, and lethal tachyarrhythmias. Diagnosis is based on measurement of plasma or urine metanephrines combined with conventional and nuclear imaging. Effective treatment is represented by surgical resection. We report a case of a 19-year-old patient with recurrent acute pericarditis; integrated imaging detected a large cardiac mass suggestive for PGL.
A 19-year-old male suffered pleuritic chest pain and fever for 4 days; electrocardiogram showed inferior ST elevation and transthoracic echocardiography a 2.2 cm pericardial effusion; these findings led to diagnose acute pericarditis. After a relapse of pericarditis, cardiac magnetic resonance and cardiac computed tomography (CCT) were performed, revealing a cardiac mass with radiological features of PGL. Blood and urine tests detected elevated levels of 3-methoxytyramine and chromogranin A. Gallium-68 positron emission tomography confirmed high metabolic activity of the mass. A negative 123-I-MIBG scintigraphy ruled out the possibility of radiometabolic treatment. A second CCT excluded the chance of surgical resection, due to intra-lesional course of the left anterior descending coronary artery. The young patient was referred to a different centre to achieve reduction of the mass, in order to potentially resect it afterwards.
Cardiac PGLs are rare tumours with significant morbidity related to norepinephrine secretion. In this case, without typical clinical manifestations and with no chance of surgical resection, integrated imaging played a central role in the differential diagnosis between PGL and other cardiac masses, providing both static and dynamic characterization.
副神经节瘤（PGLs）是起源于肾上腺外部位的罕见神经内分泌肿瘤。心脏PGLs可导致严重高血压、心悸和致命性快速心律失常。诊断基于血浆或尿液甲氧基肾上腺素的测定以及传统和核成像检查。有效的治疗方法是手术切除。我们报告一例19岁复发性急性心包炎患者；综合成像检测到一个提示为PGL的巨大心脏肿块。
一名19岁男性胸痛伴发热4天；心电图显示下壁ST段抬高，经胸超声心动图显示心包积液2.2 cm；这些表现导致诊断为急性心包炎。心包炎复发后，进行了心脏磁共振成像和心脏计算机断层扫描（CCT），发现一个具有PGL放射学特征的心脏肿块。血液和尿液检查检测到3-甲氧基酪胺和嗜铬粒蛋白A水平升高。镓-68正电子发射断层扫描证实该肿块具有高代谢活性。123-I-间碘苄胍闪烁扫描阴性排除了放射性代谢治疗的可能性。第二次CCT排除了手术切除的可能性，因为左前降支冠状动脉走行于病灶内。这位年轻患者被转诊至另一家中心以缩小肿块，以便之后有可能进行切除。
心脏PGLs是罕见肿瘤，与去甲肾上腺素分泌相关的发病率较高。在本病例中，由于没有典型临床表现且没有手术切除机会，综合成像在PGL与其他心脏肿块的鉴别诊断中发挥了核心作用，提供了静态和动态特征。
