EBV associated smooth muscle tumour

Epstein-Barr virus-associated smooth muscle tumours (EBV-SMT) are rare and encountered in immunocompromised individuals.

These tumours 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 paediatric 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.

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, lungspharynx, and larynx also relatively frequently involved 1,2.

Clinical presentation will, therefore, vary widely, depending on tumour location 1. On occasion, these tumours will be multifocal, representing simultaneous multiple primaries rather than metastases 1-3.

EBV-SMT range from indolent leiomyomas to aggressive leiomyosarcomas 1. In both cases, co-infection with Ebstein-Barr virus is necessary for the diagnosis 1

Imaging findings are non-specific, with tumours appearing as enhancing soft-tissue masses 1.

Treatment of EBV-SMT is primarily with resection, although radiotherapy and chemotherapy have also been used. In the setting of HIV/AIDS, highly active antiretroviral therapy (HAART) is also potentially indicated 1.

Prognosis and aggressiveness are variable but seem to be somewhat more favourable than conventional 'sporadic' leiomyosarcomas 1.

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rID: 51639
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