[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Pancreas](https://durgical.com/en/general-pathology/areas/pancreas/) › Acinar cell carcinoma and pancreatoblastoma

# Acinar cell carcinoma and pancreatoblastoma

- Lesion

Two rare pancreatic cancers come from enzyme-making cells: one in older adults, one in young children.

Updated Nov 12, 2026

## Key points

- Acinar cell carcinoma: older adults; solid cellular tumour with little stroma; trypsin and BCL10 positive.
- Lipase hypersecretion syndrome: metastatic fat necrosis (skin nodules, bone marrow) and arthralgia.
- Pancreatoblastoma: most common pancreatic cancer of young children; squamoid nests; AFP may be raised.
- Pancreatoblastoma: loss of 11p (with Beckwith-Wiedemann association) and APC or CTNNB1 alterations.
- Both lack the dense desmoplasia of ductal adenocarcinoma.

## 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)

## Memory aid

Acinar cancer spits lipase into the blood; pancreatoblastoma is the child's tumour with squamoid nests.

## 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).

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## Connections

## Related 3

- [Pancreatic ductal adenocarcinoma](https://durgical.com/en/general-pathology/pancreatic-ductal-adenocarcinoma/)
- [Tumours of infancy and childhood](https://durgical.com/en/general-pathology/tumours-of-infancy-and-childhood/)
- [Patterns of necrosis: coagulative, liquefactive, caseous, fat, fibrinoid, gangrenous](https://durgical.com/en/general-pathology/patterns-of-necrosis-coagulative-liquefactive-caseous-fat-fibrinoid-gangrenous/)

## Related conditions 2

- [Pancreatic cancer](https://durgical.com/en/conditions/pancreatic-cancer/)
- [Beckwith–Wiedemann syndrome](https://durgical.com/en/conditions/beckwith-wiedemann-syndrome/)

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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](https://www.cancer.gov/types/pancreatic/hp/pancreatic-treatment-pdq) (National Cancer Institute, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/acinar-cell-carcinoma-and-pancreatoblastoma/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/acinar-cell-carcinoma-and-pancreatoblastoma/