Acinar cell carcinoma and pancreatoblastoma
- Lesion
Two rare pancreatic cancers come from enzyme-making cells: one in older adults, one in young children.
What it is
Acinar cell carcinoma is a rare malignant tumour showing acinar differentiation, with zymogen granules and enzyme production. Some patients develop a lipase hypersecretion syndrome with subcutaneous and bone marrow fat necrosis and polyarthralgia. Pancreatoblastoma is a rare tumour of young children with acinar differentiation and characteristic squamoid nests; it is associated with Beckwith-Wiedemann syndrome and shows alterations of chromosome 11p and of the Wnt pathway.
Step by step
- Neoplastic cells keep acinar programmes and make zymogen granules.
- Enzymes such as lipase may be secreted into blood in large amounts.
- Circulating lipase digests fat at distant sites: skin nodules, joints, marrow.
- In children, Wnt activation and 11p changes in pancreatic progenitors produce pancreatoblastoma.
Rare pancreatic carcinomas with acinar differentiation
| Tumour | Age | Histology | Clues |
|---|---|---|---|
| Acinar cell carcinoma | Older adults | Sheets and acini of cells with granular cytoplasm and single nucleoli; little stroma | Trypsin, chymotrypsin, BCL10 positive; lipase hypersecretion syndrome |
| Pancreatoblastoma | Young children | Acinar cells with squamoid nests | AFP may be raised; 11p loss; Beckwith-Wiedemann; Wnt pathway |
Markers
- Trypsin
- Chymotrypsin
- BCL10
- Serum lipase
- Serum AFP (pancreatoblastoma)
Test yourself
Think of the answer, then tap the question to see it.
What is the lipase hypersecretion syndrome?
Release of large amounts of lipase from acinar cell carcinoma, causing fat necrosis in skin and marrow and polyarthralgia.
What histologic feature is characteristic of pancreatoblastoma?
Squamoid nests among cells with acinar differentiation.
Which syndrome is associated with pancreatoblastoma?
Beckwith-Wiedemann syndrome (chromosome 11p alterations).
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Pancreatic Cancer Treatment (PDQ®) - NCI (National Cancer Institute, 2026).
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Representative model, for educational purposes only.