title: Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis.
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 alpha-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.
authors.affiliation: Department of Pathology, Instituto Nacional de Enfermedades Neoplásicas, Lima, Peru.
authors.forename: Eduardo-Alfredo
authors.initials: EA
authors.lastname: Zevallos-Giampietri
authors.forename: Hugo Heinicke
authors.initials: HH
authors.lastname: Yañes
authors.forename: José
authors.initials: J
authors.lastname: Orrego Puelles
authors.forename: Carlos
authors.initials: C
authors.lastname: Barrionuevo
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D015658:HIV Infections / Q000150:complications*; D006257:Head / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis* / Q000209:etiology / Q000821:virology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000821:virology*