Wang Qi, Huang Zhe-Yong, Ge Jun-Bo, Shu Xian-Hong
Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai 200032, China.
World J Clin Cases. 2020 Nov 26;8(22):5707-5714. doi: 10.12998/wjcc.v8.i22.5707.
Paraganglioma is a rare disease that can be lethal if undiagnosed. Thus, quick recognition is very important. Cardiac paragangliomas are found in patients who have hypertension. The classic symptoms are the triad of headaches, palpitations, and profuse sweating. We describe a very rare case of multiple paragangliomas of the heart and bilateral carotid artery without hypertension and outline the management strategies for this disease.
A 46-year-old man presented with the chief complaint of recently recurrent chest pain with a history of hemangioma of the bilateral carotid artery that had been surgically removed. He was found to have an intracardiac mass in the right atrioventricular groove and underwent successful excision. The final pathology demonstrated that the intracardiac mass was a cardiac paraganglioma, and the patient had an increased level of normetanephrine in the blood. The pathology and immunohistochemistry results showed that the bilateral carotid masses were also paragangliomas. During the 3 mo follow-up period, the patient did not experience recurrence of chest pain.
To our knowledge, this is the first case of multiple paragangliomas of the heart and neck without hypertension. This rare disease can be lethal if left undiagnosed. Thus, quick recognition is very important. The key to the diagnosis of cardiac paraganglioma is the presence of typical symptoms, including headaches, palpitations, profuse sweating, hypertension, and chest pain. Radiology can demonstrate the intracardiac mass. It is important to determine the levels of normetanephrine in the blood. The detection of genetic mutations is also recommended. Surgical resection is necessary to treat the disease and obtain pathological evidence.
副神经节瘤是一种罕见疾病，若未被诊断，可能会致命。因此，快速识别非常重要。心脏副神经节瘤见于患有高血压的患者。典型症状为头痛、心悸和多汗三联征。我们描述了一例非常罕见的心脏及双侧颈动脉多发副神经节瘤且无高血压的病例，并概述了该疾病的治疗策略。
一名46岁男性，以近期反复胸痛为主诉就诊，有双侧颈动脉血管瘤手术切除史。发现其右房室沟有一心脏内肿物，并成功切除。最终病理显示心脏内肿物为心脏副神经节瘤，患者血液中去甲肾上腺素水平升高。病理及免疫组化结果显示双侧颈动脉肿物也是副神经节瘤。在3个月的随访期内，患者未出现胸痛复发。
据我们所知，这是首例无高血压的心脏及颈部多发副神经节瘤病例。这种罕见疾病若未被诊断可能会致命。因此，快速识别非常重要。心脏副神经节瘤诊断的关键是存在典型症状，包括头痛、心悸、多汗、高血压和胸痛。影像学检查可显示心脏内肿物。测定血液中去甲肾上腺素水平很重要。也建议检测基因突变。手术切除对于治疗该疾病并获得病理证据是必要的。
