Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: Review of literature, emphasizing the differential diagnosis and pathogenesis

Eduardo Alfredo Zevallos-Giampietri, Hugo Heinicke Yañes, José Orrego Puelles, Carlos Barrionuevo

Research output: Contribution to journalArticlepeer-review

29 Scopus citations

Abstract

We describe the clinical, radiologic, surgical, and pathologic findings of a 29-year-old Peruvian human immunodeficiency virus-infected man with a primary parasellar meningeal leiomyosarcoma involving the left lesser esphenoidal wing and the cavernous sinus. Over a period of 13 months, he developed headache, vomiting, insomnia, and diplopia. Magnetic resonance imaging revealed a left parasellar extra-axial mass that was isointense in T1, hypointense in T2, and gadolinium-enhanced. The patient underwent subtotal resection of the tumor. The neoplasm was composed of spindle cells with smooth-muscle features. It showed moderate atypia, inconspicuous nucleoli, and scanty mitosis. No tumor necrosis was detected. The immunohistochemistry revealed strong positivity for vimentin, desmin, and smooth-muscle α-actin. A low-grade leiomyosarcoma was diagnosed. The in situ hybridization showed positive nuclear reactivity for Epstein-Barr virus-encoded RNA. The immunohistochemistry was negative for Epstein-Barr virus latent membrane protein 1. The main differential diagnosis of primary meningeal smooth-muscle tumors includes meningioma and peripheral nerve sheath tumors. Epstein-Barr virus has been demonstrated in most smooth-muscle tumors associated with acquired immune deficiency syndrome (AIDS). Primary meningeal smooth-muscle tumors, exceedingly rare neoplasms, remarkably affect young adults with AIDS. Comparatively, most AIDS-related visceral (nonmeningeal) smooth-muscle tumors have been reported in children. The permissiveness and tumorigenesis associated with Epstein-Barr virus may depend on the age of human immunodeficiency virus infection.

Original languageEnglish
Pages (from-to)387-391
Number of pages5
JournalApplied Immunohistochemistry and Molecular Morphology
Volume12
Issue number4
DOIs
StatePublished - Dec 2004
Externally publishedYes

Keywords

  • AIDS
  • CNS
  • EBV
  • HIV
  • Immunohistochemistry
  • In situ hybridization
  • Leiomyosarcoma
  • Meninges
  • Smooth-muscle tumors
  • Soft tissue tumor

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