SURG-33. ROLE OF SURGERY IN TREATING EPSTEIN-BARR VIRUS-ASSOCIATED SMOOTH MUSCLE TUMOR (EBV-SMT) WITH CENTRAL NERVOUS SYSTEM INVASION: A SYSTEMIC REVIEW. (9th November 2020)

• Record Type: Journal Article
• Authors: Chen, Ko-Ting; Lau, Ka-Wei; Hsu, Yu-Wei; Lin, Yin-Ting
• Is Part Of: Neuro-oncology. Volume 22(2020)Supplement 2
• Page Start: ii210
• Page End: ii210
• Publication Date: 2020-11-09
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noaa215.879
• Languages: English
• ISSNs: 1522-8517

Abstract

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