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