Epstein-Barr virus-associated smooth muscle tumors are rare and encountered in immunocompromised individuals.
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Epidemiology
These tumors are generally exceedingly rare, and only seen with any frequency in the setting of immunosuppression, particularly in HIV/AIDS patients, but also post-transplantation and in common variable immunodeficiency syndrome. In the HIV population, they are encountered as non-AIDS-defining cancers in both adult and pediatric populations 1. The age range is therefore defined by the underlying cause of immunosuppression, but they tend to be encountered in young adults (mean age of diagnosis 25 years; range: 2.7 to 49 years) 1. No strong gender predilection has been identified 1.
Clinical presentation
Although they occur almost anywhere in the body, the central nervous system (both intra- and extra-axial) is the most common site of involvement, with the gastrointestinal tract, liver, skin, lungs, pharynx, and larynx also relatively frequently involved 1,2.
Clinical presentation will, therefore, vary widely, depending on tumor location 1. On occasion, these tumors will be multifocal, representing simultaneous multiple primaries rather than metastases 1-3.
Pathology
EBV-SMT range from indolent leiomyomas to aggressive leiomyosarcomas 1. In both cases, co-infection with Ebstein-Barr virus is necessary for diagnosis 1.
Radiographic features
Imaging findings are non-specific, with tumors appearing as enhancing soft-tissue masses 1.
Treatment and prognosis
Treatment of EBV-SMT is primarily with resection, although radiotherapy and chemotherapy have also been used. In the setting of HIV/AIDS, antiretroviral therapy (ART) is also potentially indicated 1.
Prognosis and aggressiveness are variable but seem to be somewhat more favorable than conventional 'sporadic' leiomyosarcomas 1.