Refractory pneumothorax and hemothorax associated with metastatic scalp angiosarcoma

BACKGROUND: Pulmonary metastasis of scalp angiosarcoma (SA) is a rare, but life-threatening disease, challenging to diagnose and manage. We report two cases of pneumothorax and hemothorax with pathologically proven metastasis of SA in the parietal pleura, which was not predictable from images and difficult to manage. PATIENT A: A 73-year-old man with SA underwent chemoradiotherapy and surgical resection for primary skin lesion, was sent to our department to treat right empyema, which was developed during chest tube drainage for pneumothorax. Computed tomography (CT) showed multiple bullous lesions. We performed repetitive video-assisted thoracoscopic surgery (VATS) for the debridement and hemostasis; however, hemothorax was uncontrollable. The repeated cytology of pleural effusion showed no malignancy. We eventually performed fenestration and metastatic SA was pathologically diagnosed by the biopsy of parietal pleura. The patient developed respiratory failure and uncontrolled anemia, which were fatal. PATIENT B: A 71-year-old man with SA previously treated with chemoradiotherapy was referred to our department for left pneumothorax. CT showed multiple bullous lesions at apex without any changes at parietal pleura. VATS was performed and the apex bullous lesion with air leakage was resected. The parietal pleura showed several dark-red spots and the biopsy was undertaken. The pathological diagnosis was a metastasis of SA along with visceral pleura and parietal pleura. The patient then developed right pneumothorax and left hemopneumothorax. Bilateral pleurodesis was ineffective and the patient died due to deteriorating general condition. CONCLUSIONS: In patients with a history of SA who develop pneumothorax and hemothorax, metastatic SA to visceral and parietal pleura should be always considered. Surgical biopsy, not cytology, is needed for pathological diagnosis. Lesions in the parietal pleura prior to hemothorax were thoracoscopically observed in one case. Surgeons must recognize that conventional surgical intervention or pleurodesis will have unsatisfactory results.