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.

Abstract

Epstein‐Barr virus‐associated smooth muscle tumor (EBV‐SMT) is a rare mesenchymal tumor occurred almost exclusively in immunocompromised hosts. This article provides a systematic review of literature under PRISMA guideline on clinical features, treatment modalities, roles of surgical intervention, and outcomes of all 65 reported EBV‐SMTs with central nervous system (CNS) invasion. Over 95% of reported cases were immunocompromised, while human immunodeficiency virus infection and post‐organ transplantation were the most commonly associated underlying causes (near 90%). Despite a heterogeneous follow‐up period, a 1‐year survival rate of 76.0% and 5‐year survival rate of 59.6% may support the indolent and non‐deadly nature of EBV‐SMT even with CNS invasion. Immune survey and reconstruction should be conducted for every patient with CNS EBV‐SMT. Surgical resection is mostly adopted as primary treatment to obtain diagnosis and relieve compressive effect. A total resection of tumor may be beneficial if tumor was symptomatic and had intracranial invasion.