A rare case of primary angiosarcoma of the anterior mediastinum
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CASE REPORT
A rare case of primary angiosarcoma of the anterior mediastinum Kazuhiro Yamazaki1 · Kenji Minakata1 · Takeichiro Nakane1 · Hideyuki Kinoshita2 · Kiminobu Tanizawa3 · Shinji Sumiyoshi4 · Hiroki Sato5 · Kenji Minatoya1 · Ryuzo Sakata1 Received: 12 July 2020 / Accepted: 8 November 2020 © The Japanese Association for Thoracic Surgery 2020
Abstract A 32-year-old male patient was initially diagnosed with fibrosing mediastinitis. The patient subsequently developed severe dyspnea and was further diagnosed with constrictive pericarditis due to the extent of fibrosing mediastinitis around the heart. Therefore, the patient underwent surgical resection of the fibrotic tissue in the anterior mediastinum including the pericardium and the pleura. Postoperative histological and immunohistochemical analyses revealed angiosarcoma. Seven years after the diagnosis, he is still alive. Herein, we report a case of atypical primary angiosarcoma of the anterior mediastinum causing constrictive pericarditis and restrictive pulmonary dysfunction. Keywords Angiosarcoma · Anterior mediastinum · Constrictive pericarditis · Congestive pulmonary dysfunction
Introduction
Case
An angiosarcoma is the most common primary malignant tumor of the heart with poor prognosis. However, primary angiosarcoma of the anterior mediastinum is extremely rare with a limited number of case reports. We reported a case of primary mediastinal angiosarcoma atypically causing constrictive pericarditis and restrictive pulmonary dysfunction.
A 32-year-old male physician developed worsening fatigue and severe dyspnea. He first recognized exertional dyspnea 4 years ago. One year prior to this presentation, a chest computed tomography (CT) showed bilateral pleural effusion and diffuse enlargement of the anterior mediastinum. Pleural effusion was found to be chylous, and no malignancy was observed on cytological examination. Thoracoscopic biopsy revealed inflammatory fibrous tissue of the anterior mediastinum. Thus, he was diagnosed with fibrosing mediastinitis and subcutaneous administration of sandostatin was effective against chylothorax [1]. At this point, his pulmonary function test (PFT) revealed that vital capacity (VC) and forced expiratory volume in 1 s ( FEV1.0) had decreased from 2.51 to 1.00 L and from 2.04 to 0.96 L, respectively during a 3-year interval. Arterial blood gas (ABG) analysis revealed pH 7.36, P aO2 60.8 mmHg, and P aCO2 73.7 mmHg on room air. Although noninvasive positive pressure ventilation (NPPV) was initiated with high inspiratory positive airway pressure (IPAP: 28 cmH2O), his dyspnea worsened. At the time of this presentation, a chest CT revealed thickness of the pericardium and the left pleura (Fig. 1a). Cardiac catheterization was consistent with constrictive physiology as follows: systemic blood pressure (SBP) 104/75 (80) mmHg, pulmonary artery pressure (PAP) 44/25 (32) mmHg, pulmonary capillary wedge pressure (PCWP) 24 mmHg, right atrial pressure (RAP) 26 mmHg, and cardiac index (CI)
* Kazuhiro Yamazaki yamakazu@
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