Calcifying Aponeurotic Fibroma
Synonyms: Juvenile aponeurotic fibroma
Fibroblastic tumour occuring in children and young adults
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Synonyms
Juvenile aponeurotic fibroma
Category
Soft tissue
Behaviour
Intermediate
Gender
Male
Tissue of Origin
Fibrous
Epidemiology
- Rare fibroblastic tumour with calcification
- Children and young adults most affected
Clinical Features
- Slowly growing painless mass in palm or sole
- Often adherent to tendons or fascia
- Hard consistency due to calcification
Location
- Palm of hand
- Sole of foot
- Wrist
- Ankle
Imaging
- Plain radiograph: stippled calcifications characteristic
- MRI: soft tissue mass with calcifications
Pathology
- Fibroblastic tissue with focal calcifications
- Cartilage-like differentiation around calcifications
- Palisading round cells at calcification periphery
Genetics
FN1-EGF gene fusions reported
Treatment
- Marginal or wide excision
- High recurrence rate if incompletely excised
Prognosis
- Benign/intermediate - no metastasis
- Local recurrence common (>50%), usually wihtin 3 years
Key Points
- Fibroblastic tumour occuring in children and young adults
- Seen primarily on hands and feet
- Stippled calcifications on imaging are characteristic
- High local recurrence rate
- Locally aggressive but does not metastasise
Workup - Blood Tests
None required
Workup - Local Imaging
- Plain radiograph for calcifications
- MRI for soft tissue extent
Workup - Biopsy
Excisional biopsy
Workup - Staging
No staging required
Follow-up Summary
- 1
Longer term follow-up recommended due to high recurrence rate
- 2
No specific protocol
Medical disclaimer
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