Clinical features
- 20% of all paediatric tumours
- First six months of life (90%)
- A cause of foetal hydrops
- Associated with cutaneous haemangioma (10-40%)
- Association with hypothyroidism
- Female predominance
- Association with Kasabach Merritt Syndrome
- Cardiac failure due to shunting through tumour (15%)
- Asymptomatic cases
- Spontaneously regression
- Normal serum alpha-fetoprotein
- Multicentric disease
Cytopathology
- Blood, normal hepatocytes and bile duct cells are the most common cellular element present in the smears
- Dispersed scarce plump elongated endothelial tumour cells
- Oval to spindle-shaped nuclei
- No atypia
- Scant cytoplasm
- In most cases cytology is not diagnostic or is only a suspicious result together with clinical and radiology settings.
- It is important to exclude hepatoblastoma, hepatocarcinoma and embryonal sarcoma
Immunocytochemistry
- CD34:positive
- GLUT-1: negative
GLUT-1 positive lesions classified as hepatic infantile haemangioma
Modern diagnostic techniques
- Non-contributory
Differential diagnosis
- Mesenchymal tumours in the liver- Mesenchymal hamartoma
- Other vascular tumours in the liver-
- Haemangioma, epithelioid
- Hemangioendothelioma
- Hemangiosarcoma
- Previously called as infantile hemangioendothelioma type 2
- Rare in childhood
- Rapid abdominal enlargement;
Main points
- Resection if solitary
- Involution in 6-8 months has been described
- Kasabach-Merritt syndrome may be a complication
- Death is usually due to congestive heart failure or massive bleeding