心臓傍神経節腫瘍に対する心拍動下腫瘍切除術

Off-pump Resection of Dopamine-secreting Cardiac Paraganglioma

心臓傍神経節腫瘍は非常にまれな疾患である．本邦では側方開胸心拍動下で切除を行った症例の報告はない．われわれは左房に隣接した心臓傍神経節腫瘍に対し，左側方開胸下にアプローチすることで安全性を確保しつつ，心拍動下に切除可能であった症例を経験したため報告する．

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.