Pancreatoblastoma
Pronunciation: pan-KREE-ah-toh-BLAS-toh-mah
The most common primary malignant pancreatic tumour of early childhood, characterised by a lobular architecture with squamoid corpuscles and acinar, ductal, and endocrine differentiation.
Full Definition
Pancreatoblastoma is a rare but distinctive paediatric pancreatic neoplasm accounting for the majority of primary pancreatic malignancies in children under 10 years of age. Its hallmark histological feature is the presence of squamoid corpuscles—whorled nests of squamoid cells—embedded within a background showing mixed acinar, endocrine, and ductal differentiation. It may be sporadic or arise in the context of Beckwith–Wiedemann syndrome and carries frequent abnormalities of chromosome 11p15. Serum alpha-fetoprotein (AFP) is commonly elevated and serves as a useful tumour marker. Editors should note that 'pancreatoblastoma' is a single unhyphenated word and should not be confused with 'pancreatic blastoma,' which is a less preferred synonym for the same entity, nor with hepatoblastoma, which also elevates AFP and may also occur in Beckwith–Wiedemann syndrome.
Usage
Usage note: Spell as one unhyphenated word: 'pancreatoblastoma.' Do not confuse with hepatoblastoma despite overlapping clinical features (elevated AFP, Beckwith–Wiedemann association). 'Pancreatic blastoma' is an accepted synonym but less preferred in current WHO nomenclature.
In Context
- "The resected pancreatic mass showed the characteristic squamoid corpuscles and mixed acinar–endocrine differentiation of pancreatoblastoma." — Paediatric surgical pathology report
- "An elevated alpha-fetoprotein level in a young child with a pancreatic mass should prompt consideration of pancreatoblastoma in the differential diagnosis." — Paediatric oncology case report