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Clinical features

  • Most common paediatric pancreatic tumour in infancy
  • Clinically aggressive  tumour
  • Association with Beckwith-Wiedemann syndrome
  • Age-bimodal distribution (peaks at 2 and 33years)
  • In children it can appear between 32 weeks(gestation-intrauterine) and 9 years
  • Most common in boys
  • Most frequent in the head of the pancreas
  • Serum Alpha-fetoprotein can be elevated


  • Sheets or acinar formations of cuboid cells
  • Central round nuclei with granular chromatin
  • Occasional small nucleoli
  • Moderate granular cytoplasm
  • Stromal fragments
  • Scamoid corpuscles: solid clusters of larger polygonal cells with abundant cytoplasm and clear nuclei (5) (due to the richness in biotin)


  • EMA: Positive
  • Alpha-fetoprotein: Positive
  • CEA: Positive
  • Keratins: Positive
  • Neuroendocrine markers: Positive

Modern Techniques of Diagnosis

  • Non contributory

Differential Diagnosis

  • Other small round cell tumours

Main points

  • 50% cure after excision