Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are a rare type of tumor occurring exclusively in immunocompromised patients in the setting of HIV/AIDS, post-organ transplant, and congenital immunodeficiency. These tumors require demonstration of EBV DNA on histopathologic studies in order to establish a diagnosis. The overall prognosis is good. The principle of treatment is re-establishing the host immunity, which includes antiretroviral therapy (ART) in HIV/AIDS patients and reducing immunosuppressive therapy in post-transplant patients. The role of surgery is well established when the tumor is causing a mass effect, whereas chemotherapy and radiotherapy have a limited role. Herein, we report a case of a multifocal EBV-SMT in a patient with HIV, treated successfully with standard ART along with diagnostic and therapeutic surgical resection.
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