Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome

Gary W. Mierau, Brian S. Greffe, Douglas A. Weeks

Research output: Contribution to journalArticlepeer-review

48 Scopus citations


A 11-year-old girl with common variable immunodeficiency syndrome was found to have a low-grade malignant neoplasm arising in the left temporal lobe of the brain. Ultrastructural and immunohistochemical studies established a diagnosis of leiomyosarcoma, despite the rarity of this tumor in children. In situ hybridization with the EBER probe revealed essentially all of the neoplastic cells to be infected with Epstein-Barr virus (EBV). Children with the acquired immunodeficiency syndrome (AIDS) are known to exhibit an increased incidence of smooth muscle tumors associated with EBV. Similar tumors have been reported in EBV-infected patients undergoing therapeutic immunosuppression. This appears to be the first reported case of childhood leiomyosarcoma where the cause of the underlying immunodeficiency was a genetic rather than acquired disorder. The authors conclude that electron microscopy, immunohistochemistry, and other ancillary techniques are essential in the evaluation of unusual tumors in immunocompromised children, whether the cause is hereditary or acquired.

Original languageEnglish (US)
Pages (from-to)301-305
Number of pages5
JournalUltrastructural Pathology
Issue number3
StatePublished - 1997
Externally publishedYes


  • Brain
  • Childhood
  • Epstein-Barr virus
  • Immunodeficiency
  • Leiomyosarcoma
  • Neoplasm

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Structural Biology


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