Pyothorax-associated angiosarcoma metastasized to the brain with multiple and progressively expanding hematomas: Case report and literature review

The brain metastasis of angiosarcoma is very rare, and little is known about its clinical features or therapeutic strategy. A 74-year-old male was hospitalized for disturbance of consciousness. Radiological examination revealed multiple cerebral hematomas. Gadolinium contrast-enhanced magnetic resonance imaging showed no significant enhancement at any of the lesions. To detect a suspected metastatic brain tumor or abscess, a full-body scan was performed but revealed only a poorly enhanced mass in the removal cavity caused by thoracoplasty in the left upper chest. After admission, a cascade of expansion of those hematomas occurred in the right frontal, left parietal, and right temporal lobes, and each lesion thus had to be sequentially removed by craniotomies. The pathological diagnosis of the right frontal lesion was an abscess with hematoma. However, a malignant vascular tumor was highly suspected because of many CD31(+)/Ki-67(+) cells in the left parietal lesion. A mass in the scar caused by thoracoplasty was suspected to be the primary lesion, and brain metastasis of angiosarcoma was finally diagnosed. Whole-brain irradiation and systemic paclitaxel administration were performed, and a complete response for the brain lesions was obtained for 22 months; the patient then died of an intratracheal hemorrhage. This case represents the first report of multiple brain metastases from pyothorax-associated angiosarcoma accompanied by sequentially and gradually expanding hematomas, as well as the first case with the control of metastatic brain lesions for over 1 year after the onset of neurological symptoms. Control of the lesions could be achieved by their total removal with complete hemostasis, as well as additional radio- and chemotherapy.