Posttransplant EBV-Positive Smooth Muscle Tumors in Children, Adolescents, and Young Adults: A Multi-Institution...

Epstein-Barr virus (EBV)-positive smooth muscle tumors (SMTs) are rare tumors seen in immunocompromised patients. There is no clear standard of care for the management and treatment of EBV-SMTs. Patients are often treated with chemotherapy, surgery, and/or radiation. Additional options include antiretroviral treatment, reduction in immunosuppression, and EBV-directed virus-specific T cells (VSTs). This report describes the treatment regimens and outcomes of eight patients with EBV-associated SMTs. Although no consensus treatment for EBV-SMTs has been identified, VSTs show promise in providing a period of stable disease or partial response and surgical removal may offer long-term benefits in cases of localized disease.