Spinal Epstein–Barr virus-related smooth muscle tumors: Narrative review and case study

Background: Epstein–Barr Virus (EBV) is associated with smooth muscle tumors (SMT) in immunocompromised individuals, including those with HIV or post-transplant status. EBV-related SMTs are rare in the spine, making diagnosis and treatment challenging. This study provides updated insights into their diagnosis and treatment. Methods: A formal PubMed literature review was conducted using search terms related to EBV-SMTs with screening performed based on the JBI Critical Appraisal Criteria. We included systematic reviews, literature reviews, case series, and case reports while excluding any studies that were not related to EBV-SMTs. Included literature was analyzed for epidemiology, pathogenesis, histopathology, imaging, clinical presentation, management, and prognosis, with a focus on spinal cases. Results: Between 1995 and 2023, 98 general articles were identified, 62 meeting our specific inclusion criteria. An updated treatment algorithm, including a multidisciplinary approach, was outlined. A pertinent clinical case of a 55-year-old HIV-positive female with multiple thoracic spine lesions and biopsy confirmation of EBV-SMT was presented. Following vertebroplasty, tumor excision, and antiretroviral therapy, she developed recurrent lesions four years later, requiring further treatment. Conclusion: Spinal EBV-SMTs are rare CNS tumors to consider as differential diagnosis for immunocompromised patients with spine-related pain and/or neurological deficits. Neuroimaging for lesion detection followed by biopsy confirmation using specialized staining can confirm diagnosis. Multimodal treatment with efforts at optimizing immune competency, surgical resection, and emerging therapies such as mTOR inhibitors (sirolimus) and radiotherapy have been recommended. Our comprehensive review supports ongoing research efforts using multi-institutional databases to allow for enhanced scientific insights into these rare tumors.