sirolimus and Smooth-Muscle-Tumor

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.

Topics: Bone Marrow Transplantation; Child; Epstein-Barr Virus Infections; Female; Herpesvirus 4, Human; Humans; Immunocompromised Host; Immunosuppressive Agents; Prognosis; Sirolimus; Smooth Muscle Tumor; TOR Serine-Threonine Kinases

A 27-year old caucasian male was diagnosed 2.7 years after kidney transplantation with Epstein-Barr virus (EBV)-associated smooth muscle tumors in liver and spleen. The reduction in immunosuppression and conversion from tacrolimus to sirolimus did not lead to a regression of the tumors. Additionally, the patient developed a cellular rejection of his renal allograft, which was successfully treated. A combined approach with stereotactic radiofrequency ablation (SRFA) and surgical resection was effective in the treatment of the tumors.

Topics: Adult; Epstein-Barr Virus Infections; Graft Rejection; Herpesvirus 4, Human; Humans; Immunocompromised Host; Immunosuppressive Agents; Kidney Transplantation; Liver; Male; Radiosurgery; Sirolimus; Smooth Muscle Tumor; Spleen; Tacrolimus; Treatment Outcome

Topics: Antibiotics, Antineoplastic; Epstein-Barr Virus Infections; Female; Graft Rejection; Herpesvirus 4, Human; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Transplantation; Liver Neoplasms; Middle Aged; Sirolimus; Smooth Muscle Tumor