Epstein-Barr virus-associated smooth muscle tumors in a composite tissue allograft and a pediatric liver transplant recipient

Transpl Infect Dis. 2013 Oct;15(5):E182-6. doi: 10.1111/tid.12126. Epub 2013 Aug 27.


Epstein-Barr virus (EBV) is known to establish latent infections in B-lymphocytes that can cause lymphoproliferative disorders particularly in immunocompromised patients. More recently, the development of rare EBV-associated smooth muscle tumors has been reported in transplant recipients. We herein describe 2 new cases of EBV-associated post-transplant smooth muscle tumors (EBV-PTSMT), including the first in a facial composite tissue graft recipient. Among the striking features shared by these 2 patients were their young ages, the fact that they were naïve for EBV before the transplantation, that they developed a post-transplant lymphoproliferative disorder before the diagnosis of EBV-PTSMT, and that they responded favorably to reduction of immunosuppression. Radiological and histologic features of EBV-PTSMT are shown. Finally, pathophysiology and therapeutic management of EBV-PTSMT are discussed based on a comprehensive review of the literature.

Keywords: Epstein-Barr virus; PTLD; composite tissue allotransplantation; face transplantation; post-transplant lymphoproliferative disorders; post-transplant smooth muscle tumors.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Allografts
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / etiology
  • Epstein-Barr Virus Infections / therapy
  • Epstein-Barr Virus Infections / virology
  • Facial Transplantation / adverse effects*
  • Female
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / immunology
  • Herpesvirus 4, Human / isolation & purification
  • Humans
  • Immunocompromised Host
  • Immunosuppression Therapy / adverse effects
  • Infant
  • Liver Transplantation / adverse effects*
  • Lymphoma, B-Cell / diagnosis
  • Lymphoma, B-Cell / etiology
  • Lymphoma, B-Cell / therapy
  • Male
  • Postoperative Complications / diagnosis*
  • Postoperative Complications / etiology
  • Postoperative Complications / therapy
  • Smooth Muscle Tumor / diagnosis*
  • Smooth Muscle Tumor / etiology
  • Smooth Muscle Tumor / therapy
  • Smooth Muscle Tumor / virology