Meningeal angiosarcoma: a case report and review of the literature

Primary angiosarcoma arising from the meninges is a rare form of malignant central nervous system (CNS) tumor. This study aimed to report the case of a 30-year-old female who presented with a 1-week history of worsening headache and numbness of the left limb. Neuroimaging revealed a broad based extra-axial mass occupying the right fronto-parietal area. The lesion showed a hypointense mixed intensity signal on T1 weighted image (WI) with flow voids, irregular necrotic areas, and peripheral enhancement on contrast, but a hyperintense mixed intensity signal on T2WI. Various histological patterns including sheets, small nests, cords, and sinus-like and primitive vessel-like structures were observed. Diffuse hemorrhage, necrosis, and cerebral parenchymal infiltration were also found in the tumor comprising spindle-shaped and epithelioid cells with marked atypia, abundant cytoplasm, and much of mitosis occurring. Tumor cells were positive for CD31, CD34, factor VIII (FVIII), FLI-1, CD117, ERG, and nestin. Furthermore, the MIB-1 labeling index was 10%. The patient underwent a total tumor resection with aggressive radiotherapy and chemotherapy, but had a tumor recurrence 5 months later. Compared with the previous one, the recurrent tumor from a second operation displayed almost the same morphological appearance and immune phenotype, with the MIB-1 labeling index rising up to 90%. Seven cases of meningeal angiosarcoma have been reported in the literature. A multidisciplinary treatment, such as a total tumor resection with radiotherapy or chemotherapy, is needed for primary angiosarcoma. The prognosis of patients with meningeal angiosarcoma remains poor considering the malignant nature of the tumor. It should be evaluated by more reported cases.