Giant Cell Fibroblastoma
Synonyms: Juvenile dermatofibrosarcoma protuberans
Pediatric version of DFSP; shares the same "tentacle-like" growth pattern
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Synonyms
Juvenile dermatofibrosarcoma protuberans
Category
Soft tissue
Behaviour
Intermediate
Gender
Male (3:1)
Tissue of Origin
Fibrous
Epidemiology
- Rare locally aggressive fibroblastic tumour
- Children predominately (first decade)
Clinical Features
- Painless, slow-growing, firm subcutaneous nodule or plaque
- Overlying skin may be normal or slightly discoloured
Location
- Trunk
- Inguinal region
- Extremities
Imaging
MRI: poorly defined, infiltrative subcutaneous mass. T1-isointense and T2-hyperintense with moderate enhancement
Pathology
- Spindle cells in a collagenous or myxoid stroma with multinucleated giant cells lining "pseudovascular" spaces
- CD34 positive
Genetics
Identical to DFSP: t(17;22)(q22;q13) translocation resulting in the COL1A1-PDGFB fusion gene
Treatment
Wide local excision
Prognosis
- Excellent
- Local recurrence nearly 50% if margins are inadequate
Key Points
- Pediatric version of DFSP; shares the same "tentacle-like" growth pattern
- Presence of angiectoid (vessel-like) spaces is a hallmark feature
- Requires long-term monitoring as it can recur as classic DFSP
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
MRI with contrast
Workup - Biopsy
Core needle biopsy
Workup - Staging
No staging required
Follow-up Summary
- 1
Year 1
Post-operative visit at 6 weeks. Clinical review every 6 months for the first 2 years
- 2
Year 2-5
Annual clinical checks for 5 years. MRI is generally only repeated if a recurrence is clinically suspected.
- 3
Parents should be educated on "scar checks" due to high infiltrative potential
Medical disclaimer
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