EWSR1-SMAD3-Positive Fibroblastic Tumour
Acral soft tissue tumours
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Category
Soft tissue
Behaviour
Intermediate
Gender
Female
Tissue of Origin
Fibrous
Epidemiology
- Emerging entity - very rare
- Wide age range (median age 39 years)
Clinical Features
- Small superficial acral soft tissue mass
- Slowly growing
Location
- Acral (foot most common, then hand)
- Lower limbs
Imaging
MRI: small well-defined soft tissue mass
Pathology
Spindle cell fibroblastic proliferation alternating with areas of hyalinised stroma
Genetics
EWSR1-SMAD3 fusion (t(11;15) (q24;q21))
Treatment
Wide local excision
Prognosis
- Locally aggressive - high local recurrence
- Limited data due to rarity
Key Points
- Acral soft tissue tumours
- Emerging entity in WHO 5th edition
- EWSR1-SMAD3 fusion is defining
- ERG positive - can mimic vascular tumour
Workup - Blood Tests
None required
Workup - Local Imaging
MRI with contrast of primary site
Workup - Biopsy
Excisional biopsy with molecular testing
Workup - Staging
Not required
Follow-up Summary
Medical disclaimer
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