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Epstein Barr virus associated smooth muscle tumors in the central nervous system: a case report and systematic review of the literature

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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.

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Authors and Affiliations

  1. Department of Neurological Surgery, Division of Neurosurgery at Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA, 02215-5400, USA

    V. M. Willeke & A. J. Thomas

  2. Division of Anatomic Pathology, Department of Pathology and Neurology at Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, E106, Boston, MA, 02215-5400, USA

    M. P. Anderson

  3. Hematology and Oncology, Division of Radiation Oncology, Geisinger Medical Center, 100 North Academy Avenue, Danville, PA, 17822, USA

    A. Mahadevan

  4. Department of Radiology and Division of Neuroradiology at Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA, 02215-5400, USA

    R. Rojas & R. Bhadelia

  5. Department of Surgery and Division of Neurosurgery, Hamilton Health Sciences Corp., Hamilton General Hospital, McMaster University, 237, Barton Street East, Hamilton, ON, 8L8 2X2, Canada

    E. M. Kasper

Authors
  1. V. M. Willeke
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  2. M. P. Anderson
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Correspondence to E. M. Kasper.

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Willeke, V.M., Anderson, M.P., Mahadevan, A. et al. Epstein Barr virus associated smooth muscle tumors in the central nervous system: a case report and systematic review of the literature. J Neurooncol 147, 247–260 (2020). https://doi.org/10.1007/s11060-020-03426-7

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  • DOI: https://doi.org/10.1007/s11060-020-03426-7

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