Case of primary dural epithelioid angiosarcoma with review of literature and differential diagnosis

The central nervous system (CNS) is an uncommon site for primary epithelioid angiosarcoma. We report a case of a 25-year-old male who presented with swelling over the head, headache, and weakness of the right side for 6 months. MRI revealed a heterogeneously intense large left parietal dural-based, extra-axial mass with dural tail infiltrating the brain parenchyma, overlying calvaria along with mass effect and vasogenic edema in the left parietal lobe. The patient underwent complete resection of the tumor with adjuvant radiotherapy. Histology revealed a mitotically active vasoformative neoplasm with epithelioid morphology which was immunoreactive for CD31, ERG, FLI-1, and variably for CK. Based on the histomorphological and immunohistochemical profile, a diagnosis of epithelioid angiosarcoma was rendered. The extreme rarity in this location and the highly malignant nature of this tumor makes the clinical diagnosis and management very challenging. These tumors are often considered as meningiomas on prebiopsy imaging due to dural location and dural tail. Further, the misconception may continue on histological examination if only EMA is utilized, since both meningioma and epithelioid angiosarcoma can be positive. There are only 10 previous reports of meningeal angiosarcoma reported in the literature.