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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
EBV (Epstein-Barr virus) associated smooth muscle tumors
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 4 August 2009, last major update - August 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Terminology
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● Not a WHO diagnosis
Epidemiology
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● Rare; occur in immunocompromised patients (post-transplant, AIDS)
● Adults, 60% male
Clinical
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● Often multiple tumors
● AIDS patients have multifocal tumors in liver, brain, spinal cord and adrenal gland, with slow disease progression (Oncology 2008;74;167)
● Note: EBV is also associated with leiomyosarcoma in immunocompromised (AJSP 2000;24:614)
Treatment and prognosis
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● Excision, possibly chemotherapy; also improving host immune status (Sarcoma 2008;2008:859407)
● Occasionally causes death
Micro description
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● Well differentiated smooth muscle tumors with primitive round cell areas and prominent intratumoral T cells
● No/mild atypia, no/rare mitotic figures, no pleomorphism
Micro images
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Liver tumor Fascicles of spindle cells Nuclei stain for EBER+ mRNA
with elongated nuclei EBNA-2
Positive stains
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● Actin, EBER (in situ hybridization for EBV early RNAs)
● Often desmin
Negative stains
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● HMB-45
Molecular / cytogenetics
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● Multiple tumors in same patient are clonally distinct
Additional references
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● Am J Surg Pathol 2006;30:75, Arch Pathol Lab Med 1997;121:834, N Engl J Med 1995;332:19
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > EBV (Epstein-Barr virus) associated smooth muscle tumors
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