Pancreatoblastoma : Introduction

Comments:
Introduction: Pancreatoblastoma is a rare malignant epithelial tumor of the pancreas that is usually seen in children. It accounts for 25-50% of all pancreatic neoplasms in young children.

Morphologically, it recapitulates early pancreas during embryogenesis and shows several lines of differentiation, including acinar, ductal, endocrine, as well as squamous.

Most cases are sporadic. A small percentage are associated with Beckwith-Wiedemann syndrome or familial adenomatous polyposis. The behavior is indolent and the prognosis is favorable if resected completely before it has metastasized.

This low magnification view shows tightly packed lobules separated by fibrous bands. Within the lobules, the cells are arranged in acinar structures, rosettes, solid sheets, or trabecular patterns.