Table of Contents
Definition / general | Epidemiology | Clinical features | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics descriptionCite this page: Shankar V. Epstein-Barr virus associated smooth muscle tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueebv.html. Accessed June 1st, 2023.
Definition / general
- Not a WHO diagnosis
Epidemiology
- Rare; occur in immunocompromised patients (posttransplant [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
- Excision, possibly chemotherapy; also improving host immune status (Sarcoma 2008;2008:859407)
- Occasionally causes death
Microscopic (histologic) 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
Microscopic (histologic) images
Negative stains
Molecular / cytogenetics description
- Multiple tumors in same patient are clonally distinct