Primary Pancreatic Neuroblastoma in a Neonate Presenting with Shock and Encephalopathy and Associated with Beckwith-Wiedemann Syndrome

Neuroblastoma is the most common solid tumor in children presenting in infancy. While occasionally presenting prenatally or in the neonatal period, these patients are most often asymptomatic. The adrenal gland is the most common site of origin for all neuroblastomas, and over 90% of congenital neuroblastomas arise from there. The pancreas is an exceedingly uncommon site of origin for neuroblastoma. Here we present a case of congenital neuroblastoma arising from the pancreas and presenting with shock and neonatal encephalopathy associated with clinically diagnosed Beckwith-Wiedemann syndrome.