Soft tissue tumors - EBV (Epstein-Barr virus) associated smooth muscle tumors

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Soft tissue tumors

Smooth muscle

EBV (Epstein-Barr virus) associated smooth muscle tumors (EBV-SMT)

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 23 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Not a WHO diagnosis

Epidemiology
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● Rare; occur in immunocompromised patients (post-transplant [kidney], AIDS, Arch Pathol Lab Med 1997;121:834)
● Adults, 60% male

Clinical features
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● EBV-SMT are histologically distinct from classic soft tissue smooth muscle tumors, are not readily evaluated by conventional histologic criteria; multifocal tumors are due to multiple infection events, not metastasis (Am J Surg Pathol 2006;30:75)
● 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 (Am J Surg Pathol 2000;24:614)
● EBV-SMTs have variable aggressiveness with tumor-related death occurring in 4 of 51 patients (Patholog Res Int 2011;2011:561548)

Case reports
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● 27 year old man with AIDS and tonsillar mass (Auris Nasus Larynx 2012;39:329)
● 34 year old man with AIDS and multicentric hepatic EBV associated smooth muscle tumors (Int J Clin Exp Pathol 2011;4:421)

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
● May occasionally have features of high grade sarcoma

Micro images
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Liver tumor

Tumors post-liver transplant (N Engl J Med 1995;332:19)

Liver tumors in AIDS patient

EBV-SMTs with variable aggressiveness

Positive stains
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● Actin, EBER (in situ hybridization for EBV early RNAs)
● Often desmin

Negative stains
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● HMB45

Molecular / cytogenetics description
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● Multiple tumors in same patient are clonally distinct

End of Soft tissue tumors > Smooth muscle > EBV (Epstein-Barr virus) associated smooth muscle tumors

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