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[非体外循环下切除分泌多巴胺的心脏副神经节瘤]

[Off-pump Resection of Dopamine-secreting Cardiac Paraganglioma].

作者信息

Ikuno Takeshi, Kataoka Tsuyoshi, Shiraga Kotaro, Tsuiki Mika, Naruse Mitsuhide

机构信息

Department of Cardiovascular Surgery, Kyoto Medical Center, Kyoto, Japan.

出版信息

Kyobu Geka. 2024 Sep;77(9):673-677.

Abstract

Dopamine-secreting paragangliomas is known to be rare. The average annual incidence rate was reported 0.8 per 100,000 person-years. Approximately 1 to 2% of paragangliomas occur in the chest. We describe a patient with a large dopamine-secreting cardiac paraganglioma, right adrenal tumor and carotid body tumor. A 26-year-old man with progressive exertional dyspnea was referred to our hospital for further management of multiple paragangliomas. Positron emission tomography (PET) and PET-computed tomography (CT) detected those three legions. The diameter of cardiac paraganglioma was over 45 mm and was biggest among three tumors. Firstly, therefore, we planned cardiac paraganglioma resection. Through left lateral thoracotomy in the 4th intercostal space via, cardiac paraganglioma was resected under cardiopulmonary bypass and beating heart. Postoperative course was uneventful. Tumor cells were positive for synaptophysin and chromogranin A. Free metanephrines in the serum and urinary fractionated metanephrines normalized after cardiac surgery while the other two tumors remained untreated.

摘要

分泌多巴胺的副神经节瘤较为罕见。据报道,其年平均发病率为每10万人年0.8例。约1%至2%的副神经节瘤发生在胸部。我们描述了一名患有大型分泌多巴胺的心脏副神经节瘤、右肾上腺肿瘤和颈动脉体瘤的患者。一名26岁进行性劳力性呼吸困难的男性因多发性副神经节瘤被转诊至我院进一步治疗。正电子发射断层扫描(PET)和PET计算机断层扫描(CT)检测到了这三个病灶。心脏副神经节瘤直径超过45毫米,是三个肿瘤中最大的。因此,我们首先计划切除心脏副神经节瘤。通过在第4肋间间隙经左外侧开胸,在体外循环和心脏跳动下切除心脏副神经节瘤。术后过程顺利。肿瘤细胞突触素和嗜铬粒蛋白A呈阳性。心脏手术后血清游离甲氧基肾上腺素和尿中去甲肾上腺素水平恢复正常,而另外两个肿瘤未接受治疗。

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