Qedra Naser, Kadry Mahdi, Buz Semih, Meyer Rudolf, Ewert Peter, Hetzer Roland
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany.
Ann Thorac Surg. 2009 Apr;87(4):1284-6. doi: 10.1016/j.athoracsur.2008.08.048.
Aorticopulmonary paraganglioma is a rare tumor of the middle mediastinum. Complete surgical resection is the only effective treatment, even when it may pose a surgical challenge due to the proximity of the tumor to the heart and great vessels, often rendering complete resection difficult to achieve. We report the case of a 30-year-old woman with an aorticopulmonary paraganglioma who presented with severe pulmonary hypertension due to obstruction of the pulmonary artery. In the first step, stenting of the pulmonary artery was performed and 2 months later a radical resection of the tumor using cardiopulmonary bypass under circulatory arrest and deep hypothermia was carried out. In addition, the ascending aorta and aortic arch were replaced by a prosthesis. The patient is in optimal condition and has now been disease-free for almost 7 years. We believe that this is the first description in the English literature of a successful combined management strategy in view of such an unusual manifestation of aorticopulmonary paraganglioma.
主肺动脉副神经节瘤是一种罕见的中纵隔肿瘤。完整的手术切除是唯一有效的治疗方法,即便由于肿瘤靠近心脏和大血管,手术可能面临挑战,常使完整切除难以实现。我们报告一例30岁患有主肺动脉副神经节瘤的女性病例,该患者因肺动脉阻塞出现严重肺动脉高压。第一步,先进行了肺动脉支架置入术,2个月后在循环停止和深度低温下使用体外循环对肿瘤进行了根治性切除。此外,升主动脉和主动脉弓用人工血管进行了置换。患者目前状况良好,现已无病生存近7年。鉴于主肺动脉副神经节瘤的这种不寻常表现,我们认为这是英文文献中首次对成功的联合治疗策略进行描述。