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
Role of surgery in treating epstein‐barr virus‐associated smooth muscle tumor (EBV‐SMT) with central nervous system invasion: A systemic review from 1997 to 2019
