Angiomatoid fibrous Histiocytoma
Synonyms: AFH, angiomatoid malignant fibrous histiocytoma
Can mimic haematoma or cyst clinically and radiologically
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Synonyms
- AFH
- angiomatoid malignant fibrous histiocytoma
Category
Soft tissue
Behaviour
Intermediate
Gender
M = F
Tissue of Origin
Uncertain
Epidemiology
- Rare intermediate-grade soft tissue tumour
- Peak incidence in children and young adults (1st–3rd decades)
- 0.3% of soft tissue tumours
Clinical Features
- Slow-growing subcutaneous nodule
- Can be associated with systemic symptoms: fever, anaemia, weight loss (paraneoplastic)
- Occasionally pulsatile or fluctuant mass
- Rarely causes metastasis
Location
- Extremities (limbs) most common
- Trunk
- Head and neck region
- Subcutaneous or deep dermal location
Imaging
- Well-circumscribed heterogeneous mass on MRI
- Haemorrhagic cystic spaces on T2-weighted imaging
- Pseudoangiomatous spaces not truly Vascular
- CT: well-defined soft tissue mass
Pathology
- Multinodular fibrous pseudocapsule with peripheral lymphoid cuff
- Pseudoangiomatous blood-filled spaces
- Spindle cell fascicles with histiocytic appearance
- EMA, desmin, CD99 positive; EWSR1 rearrangement
Genetics
- FET gene rearrangement
- Several translocations seen
Treatment
- Wide local excision with clear margins
- No standard systemic therapy for localised disease
- Systemic therapy considered for metastatic disease (rare)
Prognosis
- Excellent prognosis for localised disease
- Local recurrence 15%
- Metastasis rare (<5%) - to lymph node, liver and brain
- Overall survival >95%
Key Points
- Can mimic haematoma or cyst clinically and radiologically
- EWSR1 rearrangement is diagnostically useful
- Paraneoplastic symptoms resolve after excision
- Intermediate classification
Workup - Blood Tests
- FBC - anaemia if paraneoplastic syndrome present
- ESR, CRP - may be elevated in paraneoplastic context
- U&E, LFTs - preoperative baseline
Workup - Local Imaging
MRI with contrast of primary site
Workup - Biopsy
Core needle biopsy or excisional biopsy
Workup - Staging
CT chest - metastasis rare but should be excluded at staging
Workup - Other
MDT review with sarcoma specialist centre
Follow-up Summary
Soft Tissue Tumours - Localised Extremity - Low Grade
Routine MRI of primary site not indicated unless difficult to assess clinically
Paraneoplastic symptoms (fever, anaemia, weight loss) resolve post-excision - reassure patient
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