Tufted Angioma
Synonyms: Tufted angioma and kaposiform haemangioendothelioma
Rare vascular tumours of childhood
Quick Facts
Behaviour
Benign
Category
Soft tissue
Synonyms
Tufted angioma and kaposiform haemangioendothelioma
Category
Soft tissue
Behaviour
Benign
Gender
M = F
Tissue of Origin
Vascular
Epidemiology
- Infants and young children
- Associated with Kasabach-Merritt phenomenon (consumptive coagulopathy)
- Tufted angioma: more superficial; KHE: deeper and more aggressive
Clinical Features
- Skin and soft tissue vascular tumour - dull red or "port-wine" coloured plaque or nodule
- Kasabach-Merritt phenomenon: life-threatening thrombocytopenia and coagulopathy
Location
- Skin and subcutaneous tissue
- Trunk
- Extremities
- Retroperitoneum (KHE)
Imaging
MRI: infiltrative vascular lesion with ill-defined margins. T1-isointense and T2-hyperintense with internal flow voids and intense enhancement
Pathology
- Cannonball tufts of capillaries in dermis (tufted angioma)
- Spindle cells with slit-like vessels and lymphatic components (KHE)
- D2-40 positive, GLUT1 negative (distinguishes from infantile haemangioma)
Genetics
GNA14 mutations
Treatment
- Observation for most lesions
- Sirolimus (mTOR inhibitor) - most effective medical therapy
- Vincristine for Kasabach-Merritt
- Surgery rarely perfomed due to infiltrative nature and high recurrence
Prognosis
- Variable - most involute partially
- Kasabach-Merritt can be life-threatening
- Complete resolution rare for KHE
Key Points
- Rare vascular tumours of childhood
- Associated with Kasabach-Merritt phenomenon - life-threatening coagulopathy
- Sirolimus is current treatment of choice
- GLUT1 negative distinguishes from infantile haemangioma
Workup - Blood Tests
- FBC (thrombocytopenia)
- Coagulation screen (consumptive coagulopathy)
- Fibrinogen
Workup - Local Imaging
MRI primary site with gadolinium
Workup - Biopsy
Core needle biopsy
Workup - Staging
Not required
Medical disclaimer
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