Epstein-Barr virus-related smooth muscle cell tumors (EBV-SMTs) are rare albeit increasingly recognized tumors in immunocompromised patients. We report on the imaging features of EBV-SMTs occurring in the central nervous system (CNS) in two patients. Central areas of T2 prolongation, surrounding vasogenic edema, mass effect on adjacent neuroparenchyma, dural tails, and underlying bone erosions were the notable imaging findings. Besides the usual differentials of extraaxial tumors like meningioma, hemangiopericytoma, and lymphoma, CNS EBV-SMTs should be considered in immunocompromised patients.
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