Pancreatoblastoma : Clinical Features

Comments:
Clinical Features: Pancreatoblastoma is a rare pancreatic tumor that is more common in children. The age of onset is usually before 10 years (mean age at diagnosis 4-5 years). It may be seen in neonates and infants. Congenital pancreatoblastomas are frequently associated with Beckwith-Wiedemann syndrome. About 20-30% of cases occur in adults.

The presentation is non-specific and it is often discovered incidentally. Pediatric patients present with abdominal pain, abdominal mass, weight loss, diarrhea, and nausea. Rare cases present as extra-colonic manifestations of familial adenomatous polyposis in childhood. Some tumors secrete ACTH resulting in Cushing syndrome. Serum AFP is elevated in majority of cases. Some cases also show increased CEA levels.

About this image: Squamoid nests and islands are scattered among solid sheets of basophilic cells. A few rosettes and acinar structures are also seen.