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

Pediatr Blood Cancer. 2019 May;66(5):e27585. doi: 10.1002/pbc.27585. Epub 2019 Jan 6.

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.

Keywords: EBV-associated smooth-muscle tumor; Epstein-Barr virus; mammalian target of rapamycin; sirolimus.

Publication types

  • Case Reports

MeSH terms

  • Bone Marrow Transplantation / adverse effects*
  • Child
  • Epstein-Barr Virus Infections / complications*
  • Female
  • Herpesvirus 4, Human / isolation & purification
  • Humans
  • Immunocompromised Host
  • Immunosuppressive Agents / therapeutic use*
  • Prognosis
  • Sirolimus / therapeutic use*
  • Smooth Muscle Tumor / drug therapy*
  • Smooth Muscle Tumor / etiology
  • Smooth Muscle Tumor / pathology
  • TOR Serine-Threonine Kinases / antagonists & inhibitors

Substances

  • Immunosuppressive Agents
  • MTOR protein, human
  • TOR Serine-Threonine Kinases
  • Sirolimus