Infantile hemangioendothelioma

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
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