Orphanet: Pancreatoblastoma

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Disease definition

A rare neoplastic gastroenterologic disease most often found in children, which usually presents with the non-specific symptoms of a palpable mass, vomiting, abdominal pain, jaundice, and weight loss/failure to thrive. Histologically, this malignant epithelial pancreatic neoplasm of the exocrine cells is characterized by multiple lines of differentiation (acinar, ductal, mesenchymal, neuroendocrine) and the presence of squamoid nests.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Childhood, Adult
  • ICD-10: C25.1
  • OMIM: -
  • UMLS: C0334489
  • MeSH: C537162
  • GARD: 4210
  • MedDRA: -

Detailed information

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