Other tumors

Topic Completed: 1 August 2012

Minor changes: 26 October 2020

Copyright: 2002-2021,, Inc.

PubMed Search: Pancreatoblastoma[TI] full text[sb]

Deepali Jain, M.D.
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Cite this page: Jain D. Pancreatoblastoma. website. Accessed March 8th, 2021.
Definition / general
Clinical features
  • Children: 50% cured after excision; most survive and do well with chemotherapy if no metastases but those with metastases often die
  • Children: higher survival if complete resection; 5 year overall survival is 79% (Eur J Cancer 2011;47:2347)
  • Adults: mean survival 18 months
Case reports
  • 27 year old woman with breast, liver and lung metastases (JOP 2012;13:301)
Gross description
  • Partially encapsulated, often lobulated, mean 10 cm
Gross images

Images hosted on other servers:

Encapsulated heterogeneous mass with cystic components

Microscopic (histologic) description
  • Mixtures of acini, squamoid corpuscles and less commonly endocrine or ductal features
  • Very cellular, uniform epithelial cells in sheets and nests with acini / ducts
  • Squamoid corpuscles (circumscribed whorled nests of plump spindle cells with a squamous appearance and occasional keratinization) are common and specific; note: this indicates a growth pattern, not a line of differentiation
  • Pediatric cases often have hypercellular stroma, occasionally with bone / cartilage
Microscopic (histologic) images

AFIP images

Mixed solid glandular pattern

Solid tumor with scattered squamoid corpuscles

Spindle cells with sarcomatoid features

Chondroid tissue surrounded by spindle cells

Nests of squamous or epidermoid tissue

Spindle cells,
epithelial cells
and osteoid

CAM5.2+ tumor cells in acinar pattern

Tumor cells grow in acinar pattern

Images hosted on other servers:

Squamoid nests

Various images

Cytology description
Positive stains
  • Note: presence of optically clear nuclei may cause false positive immunohistochemical staining with ABC (biotin) technique
  • Pancreatic enzymes in acinar areas, CEA and mucin in luminal secretions of small acini
  • Also keratin, alpha fetoprotein (18%), aberrant (nuclear / cytoplasmic) expression of beta catenin in squamous morules
Negative stains
  • Neuroendocrine markers (rare cells may be positive)
Electron microscopy description
  • Acinar cell features
Differential diagnosis
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