Abstract
Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is a rare mesenchymal tumor that almost exclusively occurs in immunocompromised hosts. The term unknown malignant potential once used interchangeably to describe EBV-SMT and the less invasive clinical behavior than leiomyosarcoma indicate an indolent growth pattern of EBV-SMT. Nevertheless, an EBV-SMT with CNS invasion would result in neurological deficits which may jeopardize patients’ survival.We provide a systemic review of literature under PRISMA guideline on the clinical features, treatment modalities, role of surgery intervention and outcomes of all 65 reported EBV- SMTs with central nervous system (CNS) invasion. Over 95% of the reported cases were immunocompromised, while human immunodeficiency virus infection and post- organ transplantation being the most commonly associated underlying causes (near 90%). A 76.0% 1-year survival rate and 59.6% 5-year survival rate confirms the indolent and non-deadly nature of EBV-SMT even with CNS invasion. An immune survey and reconstruction should be conducted for every patient with CNS EBV-SMT. Surgical resection is mostly adopted as primary treatment to acquire diagnosis and relieve compressive effect. A total resection of tumor may be beneficial if tumor was symptomatic and had intracranial invasion