Composite Haemangioendothelioma
Rare vascular neoplasm with small risk of malignant potential
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Category
Soft tissue
Behaviour
Intermediate
Gender
M = F
Tissue of Origin
Vascular
Epidemiology
- Most common in adults
- Reported in all age groups
Clinical Features
- Slow growing, subdermal mass
- Red-blue lesion with multi-nodular appearance
- May have lymphoedema
Location
- Distal extremities (hands, feet)
- Skin and subcutaneous tissue
Imaging
MRI: multifocal enhancing soft tissue lesions
Pathology
- Combination of histological patterns: retiform, epithelioid, spindle cell
- Admixture of benign and malignant-appearing areas
Genetics
FOSB rearrangements in some cases
Treatment
- Wide local excision
- Radiotherapy for unresectable disease
Prognosis
- Low metastatic potential (15%)
- Local recurrence common
Key Points
- Rare vascular neoplasm with small risk of malignant potential
- Composite of multiple haemangioendothelioma patterns
- Wide age distribution and location
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
MRI primary site with gadolinium
Workup - Biopsy
Core needle biopsy or excisional biopsy (wide local excision)
Workup - Staging
CT chest in high risk lesions - deep, infiltrative tumours, multi-focal disease, or local recurrence
Follow-up Summary
- 1
Longer term follow-up recommended due to high recurrence rate and small malignant potential
- 2
No specific protocol
Medical disclaimer
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