SURG-80. Primary Intracranial Angiosarcoma: A Systematic Review of Scientific Literature from 2000 to 2023

Abstract OBJECTIVES To describe the clinical, radiological, and histopathological features of primary intracranial angiosarcoma, and to evaluate current management approaches and patient outcomes through a systematic review of published literature. BACKGROUND Primary intracranial angiosarcoma is an exceedingly rare and aggressive endothelial malignancy affecting the central nervous system. Due to its rarity, limited data exist to guide diagnosis, treatment, and prognostication. This study was conducted to consolidate global evidence and offer a comprehensive overview of this underreported entity. METHODOLOGY A systematic review was performed according to PRISMA guidelines. PubMed Central and Google Scholar were searched for English-language case reports and series from 2000 to 2023 using relevant keywords such as “primary intracranial angiosarcoma” and “central nervous system angiosarcoma.” Inclusion criteria were studies involving human subjects with histologically confirmed primary intracranial angiosarcoma. Data were extracted regarding demographics, clinical manifestations, radiological findings, histopathological features, treatment modalities, and outcomes. RESULTS A total of 15 studies involving 16 patients were included. The majority were adults (87.5%, n=14) with a mean age of 56.6 years, and 62.5% (n=10) were male. Hemiparesis (56.25%, n=9) and headache (43.75%, n=7) were the most common symptoms. MRI findings included mixed intensity T1/T2 signals and vasogenic edema. Immunohistochemistry showed CD31 positivity in 62.5% (n=10) and CD34 in 43.75% (n=7). Gross total resection was achieved in 56.25% (n=9). Radiotherapy was administered in 75% (n=12) and chemotherapy in 43.75% (n=7). Clinical improvement was observed in 43.75% (n=7), while 50% (n=8) deteriorated or died. CONCLUSIONS Primary intracranial angiosarcoma remains a diagnostic and therapeutic challenge due to its nonspecific presentation and aggressive behavior. MRI and histopathology are essential for accurate diagnosis. Multimodal treatment, including gross total resection and adjuvant therapy, may be associated with improved outcomes. Further multicenter research and case pooling are needed to inform evidence-based management strategies.