IntermediateSoft tissue
EBV-Associated Smooth Muscle Tumour
Synonyms: EBV-SMT
Occurs exclusively in immunocompromised patients
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Grade
Variable
Synonyms
EBV-SMT
Category
Soft tissue
Behaviour
Intermediate
Grade
Variable
Gender
M = F
Tissue of Origin
Smooth Muscle
Epidemiology
- Rare smooth muscle tumour
- Occurs in immunocompromised patients: HIV/AIDS, organ transplant, congenital immunodeficiency
- Can occur in children and adults
Clinical Features
- Multiple soft tissue masses
- CNS, liver, lung involvement common
- Immunocompromised host context
- May be multifocal across organs
Location
- CNS (intracranial)
- Liver
- Lung
- Spleen
- Adrenal gland
- GI tract
Imaging
- MRI: multiple enhancing masses
- Multiple organ involvement typical
- CNS lesions mimic other intracranial tumours
Pathology
- Spindle cells with smooth muscle differentiation
- EBV EBER in situ hybridisation positive
- SMA, desmin positive
- Variable atypia
Genetics
- EBV infection driving oncogenesis
- Clonal EBV episomal integration in tumour cells
Treatment
- Restoration of immune function (reduce immunosuppression)
- Antiretroviral therapy for HIV patients
- Surgery where feasible
- Chemotherapy options limited
Prognosis
- Variable - depends on immune status and extent of disease
- Improvement with immune reconstitution
- Multiple lesions associated with poorer prognosis
Key Points
- Occurs exclusively in immunocompromised patients
- EBV EBER positive is diagnostic
- Multiple lesions across organs is characteristic
- Immune restoration is primary treatment
Workup - Blood Tests
- FBC, U&E, LFTs
- EBV serology and PCR
- CD4 count if HIV
- Immunology screen
Workup - Local Imaging
- MRI brain
- CT chest/abdomen/pelvis
Workup - Biopsy
Core needle biopsy
Workup - Staging
PET-CT for extent of disease
Workup - Other
Immunological assessment
Medical disclaimer
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