Treatment response with sirolimus for a pediatric patient with an EBV‐associated smooth‐muscle tumor after bone marrow transplantation

Abstract

Epstein-Barr virus-associated smooth-muscle tumors (EBV-SMTs) are unique and rare neoplasms described in immunocompromised patients. The case describes a nine-year-old female with a history of acute lymphoblastic leukemia with relapse and subsequent allogeneic bone marrow transplantation who presented with multiple EBV-SMTs of the liver. EBV utilizes the mammalian target of rapamycin (mTOR) pathway for tumor growth, and sirolimus, a mTOR inhibitor, has shown to result in a short-term response. We now report an extended treatment response with sirolimus in a pediatric patient with an EBV-SMT.