Background: Pancreatoblastoma (PB) is an exceedingly uncommon pancreatic tumor arising from pancreatic exocrine cells. It is the most common malignant pancreatic tumor in childhood.
Case presentation: We report a case of PB in a 5-year-old girl who presented to our institution with severe abdominal pain, anorexia, vomiting, and jaundice. Laboratory tests were compatible with cholestasis. Ultrasound imaging showed mild intra- and extrahepatic bile duct dilation as well as a pancreatic mass. A Computed Tomography (CT) scan confirmed a large mass in the head of the pancreas, which was associated with periportal lymphadenopathy as well as anterior and inward displacement of superior mesenteric vessels. Although the mass was unresectable at the time of admission to our center, an open biopsy of the tumor was performed, which revealed a diagnosis of PB. Following six months of neoadjuvant chemotherapy, the size of the tumor was dramatically decreased, allowing the complete resection of it.
Conclusion: When a child presents with a massive solid cystic tumor in the pancreas, the possibility of pancreatoblastoma must be considered. Surgery is utilized to completely remove the tumor, while pathology and immunohistochemistry are used to confirm the diagnosis. Patients with huge tumors or extensive lymphadenopathy typically need neoadjuvant chemotherapy before surgery to downstage their disease.