Table of Contents
Pathology Research International
Volume 2011, Article ID 561548, 10 pages
http://dx.doi.org/10.4061/2011/561548
Review Article

AIDS-Related EBV-Associated Smooth Muscle Tumors: A Review of 64 Published Cases

1Department of Pathology, Presbyterian-Shadyside Hospital, University of Pittsburgh Medical Center, Pittsburgh, PA 15232, USA
2Department of Soft Tissue and Orthopedic Pathology, Armed Forces Institute of Pathology, Washington DC 20306-6000, USA

Received 6 December 2010; Accepted 1 January 2011

Academic Editor: J. Stebbing

Copyright © 2011 Bibianna Purgina et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The number of reported cases of smooth muscle tumor (SMT) arising in patients with AIDS has been increasing since the mid-1990s. The aim of this study is to characterize the epidemiology, clinical manifestations, pathologic features, prognosis and, management of Epstein-Barr virus-related SMT (EBV-SMT) in patients with AIDS. An English language literature search identified 53 articles including 64 reported cases of EBV-SMT. The majority of these reports involved patients who were young, severely immunosuppressed, and had multifocal tumors. The central nervous system was the most common site to be involved. Histologically, tumors had smooth muscle features and were immunoreactive for muscle markers and all but two tumors demonstrated the presence of EBV by either immunohistochemistry, in situ hybridization, and/or PCR. While mitoses and/or necrosis were used to separate leiomyoma from leiomyosarcoma, these features did not correlate with clinical outcome. Treatment included primarily resection, and less often radiotherapy, chemotherapy and highly active antiretroviral therapy (HAART). Overall, EBV-SMTs appear to have variable aggressiveness and clinical outcome and may exhibit a more favorable prognosis compared to conventional leiomyosarcoma. Tumor-related death from EBV-SMT occurred in only 4 of 51 patients.