Aorticopulmonary paraganglioma with severe obstruction of the pulmonary artery: successful combined treatment by stenting and surgery.

Abstract

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.