Pancreatoblastoma
Laura Webb Lamps, MD
Key Facts
Clinical Issues
Rare overall but most common malignant pancreatic neoplasm of childhood
2/3 of PB occur in children, 1/3 in adults
Mean age at diagnosis is 2.4 years in children and 40 years in adults
25-30% have elevated α-fetoprotein
Macroscopic Features
Usually solitary, well-circumscribed, lobulated neoplasm
Microscopic Pathology
Lobular growth pattern with distinct fibrous bands
Multiple histologic patterns including acinar, squamoid nests, endocrine, ductal, primitive
 The acinar pattern is one of the most common patterns of differentiation in pancreatoblastoma. |
 This gross photograph of a pancreatoblastoma shows a lobulated, well-circumscribed mass with a fleshy, whitetan cut surface. |
TERMINOLOGY
Abbreviations
Pancreatoblastoma (PB)
Definitions
Malignant epithelial neoplasm with multiple lines of differentiation
Acinar, squamous, endocrine, ductal, or mesenchymal components may be seen
Many are associated with mutations in β-catenin/APC pathway
Minority associated with SMAD4/DPC4 alterations
CLINICAL ISSUES
Epidemiology
Incidence
Rare overall but is most common malignant pancreatic neoplasm of childhood
25% of pancreatic tumors in pediatric population
Age
2/3 of PB occur in children, 1/3 in adults
Bimodal distribution; mean age at diagnosis is 2.4 years in children and 40 years in adults
Gender
No gender predominance
Ethnicity
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