Rare presentations of Epstein-Barr virus-associated smooth muscle tumor in children

Nicoleta C. Arva*, Kristian T Schafernak

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Epstein-Barr virus (EBV) has oncogenic potential and has been implicated in the etiology of a wide range of malignancies. Certain EBV-driven neoplasms, such as smooth muscle tumors (SMTs), manifest typically in immunocompromised patients. In children, these neoplasms have been encountered in the setting of primary immune disorders, specifically severe combined and common variable immunodeficiency syndromes. Human immunodeficiency virus infection and posttransplant immunosuppression, in particular liver and kidney transplantation, likewise increase the risk in the pediatric population. The location of these neoplasms appears related to the type of immunodeficiency: in acquired immunodeficiency syndrome they are frequently located intracranially or intraspinally, whereas after transplant they usually involve the liver or lung. We report 2 distinct cases of EBV-related SMT, unique through their coassociated immunosuppressive state or location: the 1st occurred in a patient with immunodeficiency secondary to NEMO gene mutation following hematopoietic stem cell transplantation; the 2nd developed in the orbit after heart transplant.

Original languageEnglish (US)
Pages (from-to)132-138
Number of pages7
JournalPediatric and Developmental Pathology
Issue number2
StatePublished - Mar 1 2016


  • Epstein-Barr virus-associated smooth muscle tumor
  • Immunosuppression
  • Transplant

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine


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