Pancreatoblastoma

Pancreatoblastoma is a rare malignant tumor of the pancreas that primarily affects young children, typically between the ages of 1 and 8 years. It is the most common pancreatic neoplasm in childhood, though it remains exceedingly rare overall. The tumor arises from the exocrine pancreas and is characterized histologically by the presence of squamoid corpuscles (distinctive nests of squamous cells) within an otherwise acinar cell differentiation pattern. Pancreatoblastoma can occur in the head, body, or tail of the pancreas and may present as a large abdominal mass. It has been associated with Beckwith-Wiedemann syndrome and familial adenomatous polyposis in some cases, and genetic alterations involving chromosome 11p (loss of heterozygosity) and abnormalities in the Wnt/beta-catenin signaling pathway have been reported. Clinically, children with pancreatoblastoma often present with a palpable abdominal mass, abdominal pain, weight loss, and failure to thrive. Some patients may have elevated serum alpha-fetoprotein (AFP), which can serve as a useful tumor marker for diagnosis and monitoring treatment response. Jaundice may occur if the tumor obstructs the bile duct. The tumor can metastasize, most commonly to the liver and regional lymph nodes, and less frequently to the lungs. The primary treatment for pancreatoblastoma is complete surgical resection, which offers the best chance for cure. When complete resection is achieved, the prognosis is generally favorable, with reported long-term survival rates exceeding 50%. For unresectable or metastatic disease, neoadjuvant or adjuvant chemotherapy regimens — often including cisplatin, doxorubicin, and other agents used in pediatric liver tumor protocols — may be employed to reduce tumor size and facilitate surgical removal. Although rare cases occur in adults, the prognosis in adult patients tends to be less favorable compared to pediatric cases.

Common questions about Pancreatoblastoma