Pneumonectomy of Primary Pulmonary Angiosarcoma with Malignant Effusion and Intrapleural Hypotonic Hyperthermic Chemotherapy: Case Report and Review of the Literature

Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: A 48-year-old male with exertional dyspnea was found to have a left massive pleural effusion. Bronchoscopic examination displayed endobronchial stenosis of the left lower bronchus B8,9,10 and diagnosed with pulmonary angiosarcoma. Chest computedtomographic scanning revealed a 5-cm mass in the left inferior lobe, which invaded the left upper bronchus. Intrapleural dissemination and malignant pleural effusion were also suspected (cT3N1M1a, c-stage IV). As a palliative initial therapy, we performed a pneumonectomy. On the 7th postoperative day, under general anesthesia, we performed video-assisted IPHHC (43 ̊C, 60 min, 200 mg/m2 of CDDP). After the IPHHC, there was no major adverse event (more than Grade 3) during the postoperative course. Histological examination of the resected specimen revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. By immunohistochemical testing, the tumor cells stained positive for markers including CD31 and the factor VIII related antigen, and the diagnosis of pulmonary angiosarcoma was made. No adjuvant chemoradiotherapy was given, and the postoperative clinical course was uneventful. Although there had been a recurrence in the chest wall, the patient eventually died twenty-one months later. Conclusion: As a new therapeutic option, we performed IPHHC after the pneumonectomy for an advanced pulmonary angiosarcoma with malignant pleural effusion. Although the patient had a recurrence in the chest Corresponding author.