MalignantSoft tissue
Sclerosing Epithelioid Fibrosarcoma
Synonyms: SEFS, Low-grade fibrosarcoma variant
Low-grade appearance but aggressive behaviour
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
- SEFS
- Low-grade fibrosarcoma variant
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
M = F
Tissue of Origin
Fibrous
Epidemiology
- Rare Low-grade fibrosarcoma variant
- Peak incidence in middle-aged adults
- Lower extremity and foot predominance
Clinical Features
- Slow-growing mass
- Often diagnosed late due to indolent appearance
Location
- Lower extremity
- Foot and ankle
- Often deep soft tissues
Imaging
MRI: infiltrative mass with Low signal (sclerotic areas)
Pathology
- Epithelioid fibroblasts arranged in cords and nests
- Abundant collagen and sclerosis
- Low mitotic rate but infiltrative
Genetics
EWSR1-CREB3L1 fusion t(7;22)
Treatment
- Wide surgical excision critical
- Chemotherapy for metastatic disease
Prognosis
- Low-grade but persistently aggressive behaviour
- High recurrence and metastatic potential
Key Points
- Low-grade appearance but aggressive behaviour
- Wide margins essential due to recurrence risk
- EWSR1-CREB3L1 fusion is diagnostic
Workup - Blood Tests
FBC, U&E, LFTs - baseline
Workup - Local Imaging
- MRI primary site - assess extent and margins
- CT chest - metastatic staging
Workup - Biopsy
- Core needle biopsy - confirm diagnosis
- EWSR1-CREB3L1 FISH - diagnostic
Workup - Staging
CT chest/abdomen/pelvis - metastases
Medical disclaimer
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