An Epstein-Barr virus-associated smooth muscle tumor successfully treated with surgical resection: a case report and literature review.

Abstract

Epstein-Barr virus–associated smooth muscle tumors (EBV-SMTs) occur predominantly in immunocompromised patients, either in the setting of solid organ transplantation or with HIV/AIDS.1 The role of EBV in the development of smooth muscle tumors was first described in the 1990s, after studies consistently showed high levels of EBV replication, EBV in-situ hybridization positivity (EBV encoded RNA [EBER]), and expression of viral genes in neoplastic cells.2-4 A small number of cases of EBV-SMTs have been reported in adults, but it is the second most common malignancy seen in children with AIDS.3-6 Typically, the disease has an indolent course, is locally invasive, and is rarely the cause of death.3,7 Treatment options include surgical resection, radiation therapy, chemotherapy, or improvement of the immune system with highly active antiretroviral therapy (HAART) in patients with HIV/AIDS. Another option in post-transplantation patients is changing the immunosuppressive therapy to sirolimus (Rapamune, Wyeth). No studies have been done to compare these treatment modalities, and a standard approach remains undefined. We report a case of an EBVSMT presenting as a superficial pelvic soft tissue mass in a patient with AIDS, which was successfully treated with surgical resection alone.