Abstract

Purpose

Epstein Barr virus (EBV)-associated smooth muscle tumors (SMT) in the central nervous system are rare tumors. EBV-associated SMT mainly occur in patient with compromised immune status. We report on a case of a HIV positive patient, who developed multiple EBV-SMTs, intracranially and in the spine. We systematically review the literature on the topic.

Case report

A 46 years old female with HIV was imaged for complaints of headaches for 2 years, when an intracranial lesion was found. The patient was followed with sequential MRI scans before an excision was performed 5 years later. Pathology revealed an EBV-associated SMT. Multiple other lesions appearing in the brain and in the spine over years were treated by stereotactic radiosurgery or by surgery. At the time of this report, the patient is alive under HARRT treatment without recurrence.

Methods

A systematic PRISMA guided literature research was conducted on the topic reviewing multiple databases for EBV-associated SMT located in brain or spine. We identified 52 patients from the literature and performed a pooled analysis.

Results

All patients in this cohort except one were immuno-suppressed from HIV, post-transplant therapy or because of CIS. Female predominance and a median age of 35 years was identified as was frequent multifocality. Therapeutic strategies varied but were mostly multidisciplinary with surgery.

Conclusion

Based on our results, EBV-associated SMT should be included in the differential diagnosis of intracranial lesions mimicking meningiomas in immuno-suppressed patients. Stereotactic radiosurgery can be offered as an alternate treatment option for suitable lesions. Long-term surveillance via MRI scanning is recommended for follow up.

中文翻译：

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爱泼斯坦巴尔病毒相关的中枢神经系统平滑肌肿瘤：病例报告和文献综述。

摘要

目的

中枢神经系统中与爱泼斯坦巴尔病毒（EBV）相关的平滑肌肿瘤（SMT）是罕见的肿瘤。EBV相关的SMT主要发生在免疫状态受损的患者中。我们报告了一名艾滋病毒阳性患者的病例，该患者在颅内和脊柱内发展了多种EBV-SMT。我们系统地回顾了有关该主题的文献。

案例报告

当发现颅内病变时，对一名46岁的HIV感染女性进行了2年的头痛投诉，并进行了成像。对该患者进行连续MRI扫描，然后在5年后进行切除。病理显示与EBV相关的SMT。多年来，通过立体定向放射外科手术或外科手术治疗了大脑和脊椎中出现的多个其他病变。在撰写本报告时，该患者在接受HARRT治疗后还没有复发。

方法

针对该主题进行了系统的PRISMA指导文献研究，该主题回顾了位于大脑或脊柱的EBV相关SMT的多个数据库。我们从文献中鉴定出52名患者，并进行了汇总分析。

结果

除一个患者外，该队列中的所有患者均因HIV，移植后治疗或CIS而受到免疫抑制。确定女性占多数，中位年龄为35岁，这是频繁的多焦点现象。治疗策略各不相同，但大多与手术有关。

结论

根据我们的结果，在免疫抑制患者中，与EBV相关的SMT应当包括在模仿脑膜瘤的颅内病变的鉴别诊断中。立体定向放射外科手术可作为适合病变的替代治疗选择。建议通过MRI扫描进行长期监视以进行随访。