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Vol. 74, No. 3-4, 2008
Issue release date: September 2008
Oncology 2008;74:167–176

Multifocal Epstein-Barr Virus-Associated Smooth Muscle Tumor in Adults with AIDS: Case Report and Review of the Literature

Gallien S. · Zuber B. · Polivka M. · Lagrange-Xelot M. · Thiebault J.-B. · Bertheau P. · Gray F. · Molina J.-M.
aDepartment of Infectious Disease, Saint Louis Hospital and University Paris 7, bDepartment of Pathology, Saint Louis Hospital, cDepartment of Pathology, Lariboisière Hospital, and dDepartment of Neurosurgery, Fondation Rothschild, Paris, France

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Objectives: The aim of this study was to provide a systematic review of Epstein-Barr virus-associated smooth muscle tumors (EBV-SMT) in human immunodeficiency virus (HIV)-infected adults, focusing on clinical and histopathologic features and outcome. Methods: A literature search was performed using Medline, Embase and the Cochrane Library. Results: We reviewed 35 cases including our case of a patient with a progressive multifocal EBV-SMT. Patients were mainly men (n = 24) with a mean age of 35.5 years. Median CD4 count was 21/mm3. Main locations were brain (n = 12), liver (n = 8), spinal cord (n = 7) and adrenal gland (n = 6). The tumors were multifocal in 34% of cases, whereas analysis of clonality showed different clones in tumors from different sites. Treatment included removal surgery in 17 cases and/or radiotherapy in 9 and therapeutic abstention in 4. Mean follow-up after diagnosis was 12.3 months. Nine patients died during this period essentially from opportunistic infection and only 2 from the disease. Conclusion: EBV-SMT should be added to the list of virally induced tumors in severely immunocompromised HIV-infected adults. Multifocality of independent tumor clones, especially in liver, brain, spinal cord and adrenal gland, and a slow disease progression seem to be the key features of these tumors, the treatment of which remains poorly defined.

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