Pancreatoblastoma

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

Cytopathology

• 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)

Immunocytochemistry

• 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