Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

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
=========================================================================

● Not a WHO diagnosis

Epidemiology
=========================================================================

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

Clinical features
=========================================================================

● 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
=========================================================================

● 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
=========================================================================

● Excision, possibly chemotherapy; also improving host immune status (Sarcoma 2008;2008:859407)
● Occasionally causes death

Micro description
=========================================================================

● 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
=========================================================================



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
=========================================================================

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

Negative stains
=========================================================================

● HMB45

Molecular / cytogenetics description
=========================================================================

● Multiple tumors in same patient are clonally distinct

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


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).