Metastatic Angiosarcoma Presenting as Ischemic Anterior Circulation Stroke

Angiosarcoma is a rare malignant neoplasm that can arise from vascular endothelium. We report a case of angiosarcoma that presented as thromboembolic stroke, review the current literature, and discuss the management challenges.

A 77-year-old man presented with a right anterior circulation stroke with a history of multiple, recent transient ischemic attacks. The diagnosis was confirmed on computed tomography and subsequent investigation revealed thrombus within the distal common carotid artery, with an atherosclerotic plaque causing a significant stenosis of the origin of the internal carotid artery above this. In the context of the presentation with recurrent events, carotid endarterectomy was recommended and subsequently performed. At the time of surgery, the common, internal, and external carotid arteries had macroscopic evidence of atherosclerotic disease but appeared otherwise normal. Intraoperatively friable thrombus adherent to the common carotid endothelium was retrieved and sent for histologic assessment. Subsequent immunohistochemistry evaluation revealed changes pathognomic with angiosarcoma.

It is unclear in this case whether the pathology originated in the carotid artery or was the result of embolization from a proximal source. There was no extraluminal carotid pathology identified at the time of surgery, and no abnormalities identified on cross-sectional imaging. Similarly, there was no evidence of a proximal source that may have resulted in tumor embolization. As such, it has proved impossible to define an optimal therapeutic pathway for this patient.