Primary Central Nervous System Angiosarcoma

• Published in: World neurosurgery 2019-12, Vol.132, p.41-46
• Main Authors: Gao, Mengqi, Li, Pengfei, Tan, Chunyu, Liu, Jiawei, Tie, Xinxin, Pang, Chao, Guo, Zongze, Lin, Yi
• Format: Article
• Language: English
• Subjects: Aged; Angiosarcoma; Brain Neoplasms - complications; Brain Neoplasms - diagnostic imaging; Brain Neoplasms - pathology; Cerebral Hemorrhage - diagnostic imaging; Cerebral Hemorrhage - etiology; Frontal Lobe - diagnostic imaging; Frontal Lobe - pathology; Hemangiosarcoma - complications; Hemangiosarcoma - diagnostic imaging; Hemangiosarcoma - pathology; Humans; Magnetic Resonance Imaging; Male; Primary central nervous system tumor; Surgery
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2019.08.128

Primary angiosarcoma of the brain is an extremely rare malignant tumor that that arises from vascular endothelial cells of the brain or meninges. The presentation, characteristics, and prognosis of this disease are not well understood. Here we report such a case. A 68-year-old Chinese man presented with confusion and progressive impairment of right limb movement. Magnetic resonance imaging (MRI) revealed a hemorrhagic lesion in the left frontal lobe. Exploratory surgery revealed a hematoma-like lesion and extensive superficial hemosiderin deposition. The postoperative pathological diagnosis was angiosarcoma. The tumor recurred at 1 month after the operation, with extensive tumor regrowth in the left hemisphere. The patient died after 4 weeks. Although accurate preoperative diagnosis of angiosarcoma of the brain is difficult, radiologists and neurosurgeons need to be aware of this rare entity. The prognosis of intracranial angiosarcoma is heterogenous. Intraoperatively, the presence of extensive superficial hemosiderosis may aid in its diagnosis.